Journal of Pediatric and Adolescent Gynecology

Three Symptomatic Cases of Myoma Uteri in Adolescence, One of Which Is STUMP tumor

Uterine leiomyomas are rare in the adolescent age group, with smooth muscle tumors of unknown malignant potential (STUMPs) being even rarer in young patients. Three cases aged 19 years, 2 with abnormal uterine bleeding and 1 with pelvic pain, were admitted to the hospital. Uterine myomas measuring 5, 7, and 12 cm were detected in the patients. Myomectomy was performed in patients with complaints resistant to medical treatment. Pathologic examinations revealed that one of the complaints was a STUMP, which is rare in the adolescent and young age group. The other 2 examinations revealed benign uterine fibroids. Although uterine myomas are quite rare in the adolescent age group, they should be considered in patients with pelvic pain, pelvic mass, and abnormal uterine bleeding. In this age group, uterine myomas and even STUMPs should be included in the differential diagnosis.

A Case of Paratubal Serous Borderline Tumor Driven by a Somatic BRAF Mutation in an Adolescent Patient

We describe a rare paratubal serous borderline tumor occurring in an adolescent and provide insight into its molecular underpinnings. A 14-year-old girl presented with irregular menstrual cycles and a large right pelvic mass. Computed tomography imaging revealed a cystic neoplasm arising from the right ovary with peripheral calcification. During laparotomy, a cystic tumor located at the right parametrium independent of the fallopian tube was identified. The ovary was intact and the tumor was successfully removed. Intraoperative diagnosis using the frozen section technique and subsequent pathology revealed a paratubal serous borderline tumor. Molecular analyses revealed a chromosomally stable tumor genome and a pathogenic somatic mutation (c.1799 T > A, p.Val600Glu) in the B-Raf proto-oncogene, serine/threonine kinase (BRAF) gene. This case shows that the BRAF p.Val600Glu mutation likely acts as an oncogenic driver in this rare neoplasm.

Effect of Surgical Specialty on Management of Adnexal Masses in Children and Adolescents: An 8-Year Single-Center Review

Children with adnexal masses might be managed by pediatric surgeons, urologists or gynecologists, with the potential for different management strategies between specialties. In this study we compared ovarian conservation rates and surgical approach for adnexal masses in children and adolescents managed either by pediatric surgeons/urologists or gynecologists at a tertiary care institution. Retrospective cohort review. Tertiary pediatric and adult university hospital. Patients younger than 18 years of age with an adnexal mass managed surgically with removal of histologically confirmed ovarian or fallopian tube tissue from 2008 to 2015. Laparoscopic or open procedure for adnexal mass. The primary outcome was rate of ovarian conservation relative to surgical specialty. The secondary outcome was surgical approach relative to surgical specialty. Forty-eight patients underwent surgery for adnexal masses; 26 (54%) under pediatric surgery/urology and 22 (46%) under gynecology care. Laparoscopy was performed in 5 (19%) pediatric and 19 (86%) gynecology cases (P = .000006). Of 24 patients older than 12 years of age with a benign tumor, 10 (42%) underwent procedures resulting in loss of an ovary with or without fallopian tube; 8 of these (80%) were under pediatric care. Of the remaining 14 (58%) who underwent ovarian conserving surgery, 12 (80%) were under gynecology care (P = .0027). Patients with a benign tumor were significantly more likely to undergo ovary-preserving surgery under gynecology care than under pediatric surgery/urology care. A multidisciplinary team approach involving gynecology and pediatric surgical specialties would be valuable in assessing the merits of ablative or conservative surgery in each case.

A Rare Case of a Giant Vulval Fibroepithelial Stromal Polyp

A fibroepithelial stromal polyp is an uncommon benign pathology of the female genital tract. Rarely, these benign tumors present as a giant lesion and are clinically difficulty to distinguish from other pathologies. A 19-year-old female presented with a vulval fibroepithelial stromal polyp measuring ∼8 × 8 × 3 cm that extended from the right labia majora. Present for 2 years, the lesion caused mild discomfort and aesthetic dissatisfaction due to its size but otherwise had no associated symptoms. It was diagnosed using a combination of imaging techniques including ultrasound and magnetic resonance imaging and histological analysis. It was removed surgically and has had no recurrence. The consideration of both benign and malignant pathologies is paramount when differentiating vulval masses. Thorough clinical reasoning and appropriate use of imaging modalities and histological analysis are essential.

Topical Hemostatic Agent May Be Mistaken for Ovarian Teratoma

Ovarian teratomas are common, as is use of topical hemostatic agents in ovarian surgery. Following laparoscopic right ovarian cystectomy, a flowable hemostatic agent was placed in the ovarian bed. Postoperative ultrasound demonstrated an enlarged heterogeneous right ovary with solid and cystic components interpreted as residual or recurrent teratoma. Visual inspection during repeat laparoscopy revealed an irregularly shaped, enlarged right ovary with a smooth cortex. A cavity inside the ovary contained brown, shaggy material. Pathological evaluation demonstrated normal ovary and fibrin. We report the first case of a hemostatic agent routinely used in ovarian cystectomy postoperatively, mimicking a teratoma. Consideration of this finding is important when planning surgery in adolescent patients to optimize fertility preservation and minimize ovarian follicle damage.

Polish Mothers and Cervical Cancer Prophylaxis: What Do They Know and What Attitude Do They Have Toward Human Papillomavirus Vaccination?

The aim of the study was to assess the knowledge and awareness of cervical cancer primary and secondary prophylaxis among our patients' mothers, and their attitude toward human papillomavirus (HPV) vaccination. During patients' appointments at Children's Memorial Health Institute Pediatric and Adolescent Gynecology Clinic in Warsaw we asked 234 mothers (aged <20-60) to complete a self-prepared survey. The data were analyzed using t-student test and chi-square test. Only 61.5% of respondents correctly indicated the purpose of cervical cytology. Similarly, 67.9% claimed that it should be performed annually, and 79.4% that till the end of life. Although 87.6% of answerers have heard about HPV vaccination, still 69.3% of them were willing to get more information. Physicians were considered the most reliable source of knowledge about HPV vaccination and had a relevant impact on mothers' decision to vaccinate (P < .05). Nearly all respondents (92.7%) weren't vaccinated against HPV. Still, 57.3% declared willingness to vaccinate their children. The vaccine skeptics indicated that they had too little information (59%), feared side effects (45%), and doubted HPV vaccine effectiveness (23%). Mothers aged 40 and older and with higher education had greater knowledge about cervical cancer screening (P < .05) and were more willing to vaccinate their children (P < .05). Since cervical cytology was invented 100 years ago, knowledge about this screening test is low. The HPV vaccine is well-recognized and well-accepted, but most respondents lack information. Consequently, it is crucial to initiate multidirectional educational actions to raise awareness about cervical cancer and its prophylaxis.

Application of Hysteroscopy in the Treatment of Mullerian Adenosarcoma of the Cervix in an Adolescent Girl with 7 Years of Follow-up

Mullerian adenosarcoma of the cervix is a rare biphasic tumor composed of a benign epithelial component and a malignant stromal component. Here, we report a rare case of Mullerian adenosarcoma of the cervix in an adolescent girl treated with hysteroscopic resection. A 16-year-old girl presented to the Affiliated Hospital of North Sichuan Medical College in April 2017 with a 1-year history of a painless vaginal mass. Ten hours before presentation, she had experienced rapid enlargement of the vaginal mass and mild vaginal bleeding. Sixteen hours after hospitalization, a mass measuring 14 × 10 × 4 cm was spontaneously expelled from the vagina. Histopathological examination of the mass confirmed a diagnosis of Mullerian adenosarcoma. On May 10, 2017, the patient underwent hysteroscopic resection of a cervical lesion and partial cervical resection. After nearly 7 years of follow-up, the tumor has not recurred. Mullerian adenosarcoma of the cervix is difficult to diagnose in adolescents. For young women affected by low-risk early-stage Mullerian adenosarcoma of the cervix, fertility-preserving treatment using hysteroscopic resection with robust follow-up is a reasonable management option.

From Storage to Survivorship: A Scoping Review of Young Adult Cancer Survivors’ Experiences and Preferences in Reproductive Survivorship Care After Fertility Tissue Preservation

Atypical Presentation of Granulosa Cell Tumor in an Adolescent: A Case Report

Granulosa cell tumors (GCTs) frequently present with elevated levels of estrogen and inhibin. Most diagnoses in the pediatric and adolescent population are juvenile-type GCTs; adult-type GCTs in this population are rare. We describe a 14-year-old female who presented with a large adnexal mass and clinical hyperandrogenism. Laboratory evaluation revealed elevated levels of free and total testosterone, low-normal estradiol, and mildly elevated alpha-fetoprotein (AFP). Other tumor markers, including inhibin, were within normal limits. Intraoperative assessment with unilateral oophorectomy, pathology, and imaging resulted in a diagnosis of a stage IA adult-type GCT. GCTs often result in elevated estrogen and inhibin B levels; however, this case demonstrates that non-classic elevations in testosterone and normal inhibin levels should not eliminate concern for a GCT, particularly in the setting of a large ovarian mass.

The Alexis Laparoscopic System as Minimally Invasive Surgical Technique in Operative Management of Huge Cystic Ovarian Tumors in Children and Young Adolescents: A Case Report

The use of the Alexis wound protector-retractor (AWPR) could further improve the laparoscopic techniques for safely removing huge ovarian tumors in children. A 15-year-old patient presented to our emergency department with a history of weeks of persistent abdominal pain. Clinical examination and magnetic resonance imaging demonstrated a 25 × 21 × 21 cm cystic mass arising from the left ovary. An en block unilateral oophorectomy and salpingectomy with the use of a medium-sized AWPR was performed without complications. The patient was discharged on the second postoperative day. The AWPR is a safe and effective tool in treating such children and adolescents with huge ovarian tumors, preserving fertility, minimizing the postoperative pain and hospital stay, and providing an excellent cosmetic outcome.

Recurrence Rates for Pediatric Benign Ovarian Neoplasms

To examine the recurrence rates of pediatric benign ovarian neoplasms METHODS: A retrospective review of females up to 21 years of age who underwent surgery for a benign ovarian neoplasm at 8 pediatric hospitals from January 2010 through December 2016 was conducted. Data include primary operation details, follow-up imaging, and reoperation details. Four hundred and twenty-six females were included in our cohort, with a median age of 15 years at the time of the primary operation. Of the patients, 69% had a mature teratoma, 18% had a serous cystadenoma, and 8% had a mucinous cystadenoma. Two-thirds of patients underwent ovarian-sparing surgery. There were 11 pathologically confirmed recurrences (2.6%) at a median follow-up of 12.8 months. The pathologically confirmed recurrence was 10.5 per 100 person-months at 12 months (SE = 5.7) for mucinous cystadenomas and 0.4 months (SE = 0.4) for mature teratomas (P = .001). For half of the patients, the pathologically confirmed recurrences occurred by 12.8 months, and for 75%, they occurred by 23.3 months. There were no differences in reoperation or recurrence on the basis of initial procedure (ovary-sparing surgery vs oophorectomy). We measured the pathologically confirmed recurrence rate for pediatric benign ovarian neoplasms in a large cohort. Oophorectomy was not protective against recurrence. Mucinous cystadenomas were at a greater risk of pathologically confirmed recurrence.

Sonographic Predictors of Ovarian Torsion in Premenarchal Girls

To identify preoperative transabdominal sonographic predictors of surgically confirmed ovarian torsion (OT) in premenarchal girls METHODS: We conducted a retrospective case-control study of 32 premenarchal girls aged 0-12 undergoing surgery for OT (cases) or a non-torsed ovarian mass (controls) from 2006 to 2017 at a single academic center. Cases had ICD-9/10 codes for torsion of the ovary, adnexa, ovarian pedicle, or fallopian tube and surgically confirmed OT; controls had codes for ovarian mass or cyst and surgically confirmed absence of OT. Preoperative transabdominal ultrasounds were analyzed by 3 radiologists blinded to final diagnosis. We used χ From 2016 to 2017, 32 patients presented with acute abdominal pain or symptoms concerning for ovarian mass requiring ultrasound imaging and subsequent diagnostic laparoscopy; 24 (75.0%) had confirmed OT by laparoscopy, and 8 (25.0%) did not. The mean age in both groups was similar (7.3 ± 2.9 years). Preoperative sonographic variables significantly associated with OT included presence of a simple cyst (20.8% vs 12.5%), ovarian heterogeneity (100% vs 12.5%), presence of peripheralized follicles (70.8% vs 0%), and asymmetry of color Doppler (75.0% vs 37.5%; all P < .05). Presence of free fluid, arterial color Doppler, and a whirlpool sign were not predictive of OT. In premenarchal patients, although certain variables on transabdominal sonography predicted surgically confirmed OT, only the presence of peripheralized follicles was unique to girls with OT. The decision to proceed with diagnostic laparoscopy for suspected OT can be aided by these specific sonographic findings but should ultimately be based on high clinical suspicion.

Imaging Appearance of Nongerminoma Pediatric Ovarian Germ Cell Tumors Does Not Discriminate Benign from Malignant Histology

Pediatric ovarian neoplasms with imaging appearance suggestive of teratoma are often presumed to have low risk of malignancy. We assessed the pre-operative imaging appearance of pediatric malignant ovarian germ cell tumors (MOGCT) and the presence of associated teratoma in a series of MOGCT. Retrospective review of clinical and pathology data. Multicenter trial for extracranial malignant germ cell tumors in young female individuals by the Children's Oncology Group (COG study AGCT0132) that included yolk sac tumor, embryonal carcinoma and choriocarcinoma. Female individuals 0-20 years of age at enrollment with ovarian primary nonseminomatous malignant germ cell tumors. Review of data forms, including prospectively collected surgical checklist documenting imaging characteristics of the tumor, and review of pathology reports. Description of imaging appearance and frequency of mixed histology with benign teratoma elements. A total of 138 female individuals (11 months to 20 years of age) had primary ovarian tumors. Imaging appearance and pathology information were available for 133 patients. Among the 133 patients, tumor appearance was solid (10.5%), solid with calcification (3.0%), mixed cystic and solid (58.7%), mixed cystic and solid with calcification (24.8%), and unknown (3.0%). In all, 54% had elements of teratoma in addition to malignant histology. Mixed cystic and solid appearance with or without calcification was seen in 83.5% of pediatric ovarian malignant germ cell tumors. Associated benign teratoma was common. The presence of a mixed cystic and solid appearance on preoperative imaging should not dissuade the surgeon from obtaining preoperative serum markers and undertaking complete surgical staging.

Successful Treatment of Nongestational Choriocarcinoma in a 15-Year-Old Girl: A Case Report

Nongestational choriocarcinoma is a rare ovarian malignancy with a prognosis worse than that of gestational choriocarcinoma. Debulking surgery is the primary treatment for ovarian carcinoma. However, fertility preservation is important in young women. A 15-year-old girl with no sexual experience was admitted for abnormal uterine bleeding. Ultrasonography showed a solid mass in the right ovary and her serum β-human chorionic gonadotrophin levels were markedly elevated. We performed right oophorectomy, omentectomy, and peritoneal washing cytology. The uterus and left adnexa were preserved. She was diagnosed with nongestational choriocarcinoma, stage IIA. She received adjuvant chemotherapy (etoposide, methotrexate, actinomycin, cyclophosphamide, and oncovin regimen) and has been disease-free for more than 5 years. Fertility-sparing surgery combined with chemotherapy is an acceptable treatment option for young patients with locally advanced nongestational choriocarcinoma.

Clinical and Computed Tomographic Features of Ovarian Lesions in Infants, Children, and Adolescents: A Series of 222 Cases

To investigate the clinical and computed tomography (CT) characteristics of ovarian lesions in infants, children, and adolescents. A retrospective analysis of the clinical and CT data was performed in 222 patients who were 20 years or younger with ovarian lesions. Patients' age, medical history, symptoms, tumor marker levels, and CT imaging findings were recorded. None. Identification of the clinical and CT features of ovarian lesions in infants, children, and adolescents. A total of 136 patients had abdominal pain, and 73 patients had palpable abdominal mass. The β-HCG was elevated in 4 and AFP was elevated in 16 of the 222 cases. A total of 235 lesions were found in 222 cases, including 75 non-neoplastic and 160 neoplastic lesions. Ovarian cyst exhibited homogeneous low density. The torsion of a normal-sized ovary demonstrated mild or no enhancement. The torsion associated with an ovarian mass demonstrated a thickened, hyperdense wall. Mature teratoma presented as a cystic mass, with bulk fat and coarse calcification. Immature teratoma appeared as a solid mass with foci of fat and fine calcification. Yolk sac tumor was shown as cystic-solid mass with intense enhancement of solid component. Wall and septation of benign epithelial tumors were relatively uniform in thickness; mural nodule was detected in borderline tumor; and malignant epithelial tumor was predominantly a solid mass with intense enhancement. Ovarian cyst is the most common non-neoplastic lesion. Torsion of a normal-sized ovary was the second most common non-neoplastic lesion, almost always causing abdominal pain. Germ cell tumor has the highest incidence among neoplastic lesions. Fat and calcification are highly specific for germ cell tumor. The elevation of AFP and HCG levels in serum indicates germ cell tumor. Ovarian epithelial tumor is usually large, benign, and predominantly cystic. The combination of clinical and imaging features is helpful for correct diagnosis.

A Common Tumor with an Unusual Finding: Mature Cystic Teratoma Containing a Ganglioneuroma

Ganglioneuromas (GNs) arise from peripheral or central areas of the autonomic nervous system, being benign lesions of neuroblastic origin. A 19-year-old Greek female patient was admitted to our clinic with symptoms of periodic lower abdominal pain and irritation since some months ago. Ultrasonography revealed a mass of 72 × 68 mm originating from the left ovary giving the characteristics of a typical dermoid cyst. Laparoscopic ovarian cystectomy was done. The histological diagnosis revealed a mature cystic teratoma with a small hidden GN. Histology remains the gold standard for their confirmation. The coexistence of GN within a mature cystic teratoma happens extremely rarely. This is one of the few cases reported in the literature.

Management of Large Pediatric and Adolescent Ovarian Neoplasms with a Leak-Proof Extracorporeal Drainage Technique: Our Experience Using a Hybrid Minimally Invasive Approach

In a pediatric setting, laparoscopic management of large cystic ovarian neoplasms with low malignancy probability is not suitable, because of the mass size; nevertheless, an effort should be made to be as minimally invasive as possible, without violating the principles of oncologic surgery. We describe our experience in managing these neoplasms with leak-proof extracorporeal drainage through mini laparotomy, followed by cyst excision or oophorectomy. Case series study, describing interventions and outcomes. Department of pediatric surgery in a tertiary pediatric and adult university hospital. Pediatric patients affected by large cystic ovarian mass. Hybrid minimally invasive approach using leak-proof extracorporeal drainage. Data on demographic characteristics, tumor marker values, and imaging findings were collected and analyzed. Outcome of surgical technique was evaluated and reported. Between 2011 and 2018, 17 patients (mean age, 10.2 years; range, 2-14 years) affected by large cystic ovarian mass, were eligible for this technique. All patients had negative preoperative tumor markers. Of the seventeen subjects, 13/17 girls (76%) underwent pelvic magnetic resonance imaging. No sign of lymphadenopathy or metastasis was found. Surgery was successful in all patients, with ovarian preservation in 5/17 cases (29.4%). Mean surgical time was 98 minutes; no intra-abdominal leakage of neoplasm content or postoperative complications occurred. Mature cystic teratoma was the most frequent histopathological diagnosis (71%). After a thorough patient selection, the management of large cystic ovarian neoplasms with leak-proof extracorporeal drainage performed through a mini laparotomy is a feasible and safe approach, with excellent cosmetic results. When achievable, ovarian-sparing surgery has to be considered.

Homemade Glove Retrieval Bag in Laparoscopic Complete Excision of Benign Ovarian Teratoma with Ovarian Tissue Preservation in Children: A Case Series

To present our experience of laparoscopic resection of pediatric benign ovarian teratomas with gonadal preservation, using a homemade glove retrieval bag. DESIGN, SETTING, PARTICIPANTS, INTERVENTIONS, AND MAIN OUTCOME MEASURES: Review of all girls with benign ovarian teratomas who were managed with laparoscopic ovarian-sparing surgery (OSS) at our hospital between January 2013 and December 2018. Eleven patients were included for analysis with a mean age of 6.1 years. Ten patients received elective surgery, whereas 1 patient received emergency surgery because of ovarian torsion. Main indication for OSS was the existence of a dissection plane between tumor margins and healthy ovarian tissue. Postoperative outcome and follow-up were uneventful with a median follow-up of 30.1 months (range; 12-60 months). Laparoscopic OSS can be safely performed for these tumors. Laparoscopic magnification with energy devices are excellent tools in such procedures. The homemade glove bag can be used to retrieve the tumor effectively in countries with limited resources.

Ovarian Sex Cord Stromal Tumor, Steroid Cell, NOS in an Adolescent: A Case Report

Ovarian steroid cell tumor, not otherwise specified (NOS), is a rare type of sex cord stromal tumor, which often presents with androgenic symptoms and has a high frequency of malignancy. This is a case of a 14-year-old Native American girl who presented with acne, amenorrhea, and virilization and was found to have a 2.9-cm solid ovarian mass. Initial pathology revealed steroid-appearing cells with round nuclei, clear/vacuolated cytoplasm, and a low mitotic index. Final diagnosis was ovarian steroid cell tumor, NOS Stage IA. A laparoscopic left salpingo-oophorectomy was subsequently performed. No tumor recurrence was noted 2 years after her initial diagnosis. Long-term data on these tumors are limited; however, malignancy, recurrence, and death have been reported. This suggests that close follow-up is essential for appropriate management.

Small Cell Carcinoma of Ovary, Hypercalcemic Type: Cytologic, Histopathologic, and Immunohistochemical Landscapes of a Rare Case

Small cell carcinoma of ovary, hypercalcemia type (SCCOHT), also known as the malignant rhabdoid tumor of the ovary, is a rare and highly aggressive malignancy affecting younger women. The pathogenesis involves mutations in SWI/SNF-related, matrix-associated, actin-dependent regulator chromatin group A4 (SMARCA4)/Brahma-related gene 1 (BRG1) and/or SWI/SNF-related, matrix-associated, actin-dependent regulator chromatin group A2 (SMARCA2)/Brahma homolog (BRM). A 10-year-old girl presented with lower abdominal pain and a mass for the past 2 weeks. She underwent ultrasound-guided fine needle aspiration and core needle biopsy from the pelvic mass followed by surgery. On the basis of the characteristic morphologic and immunohistochemical features, a diagnosis of SCCOHT was rendered. Chemotherapy was started, however, she succumbed to the disease.

Metachronous Contralateral Cystadenoma After Ovariectomy for Juvenile Granulosa Cell Tumor in a Young Girl: An Uncommon Association

A unique Case of metachronous contralateral cystadenoma diagnosed 2 years after a juvenile granulosa cell tumor (JGCT) of the ovary is reported. One year after a left ovariectomy for JGCT, a 7 year-old girl was found to have, during her standard ultrasound follow-up, a 18-mm cyst in the right ovary, which was initially considered to be a follicular cyst. Six months later, the mass appeared to be increased and characterized by multivacuolar features, and inhibin A was mildly elevated. A laparoscopic-assisted cystectomy was performed, sparing healthy ovarian tissue around. The pathology report showed a benign mucinous cystadenoma.

A Huge Ovarian Mucinous Cystadenoma in a 15-Year-Old Girl with Situs Inversus Totalis: Report of a Rare Case

Ovarian mucinous cystadenomas with situs inversus totalis are infrequent in pubertal girls. Surgical techniques on their treatment without affecting ovary anatomical and physiological function have always been a great challenge. A 15-year-old girl presented with abdominal distension and pain due to some huge growths. Computed tomography imaging showed that the heart and whole abdomen viscera were inversely located. Two big low-density masses were found in the abdominopelvic cavity. An exploratory laparotomy was performed and 2 tumors were removed. Pathology confirmed a mucinous cystadenoma. Ovarian mucinous cystadenomas with situs inversus totalis can be detected with detailed physical and radiological examination. For adolescent female patients, particular attention should be paid to protect the reproductive anatomical structure during surgery.

Recurrent High-grade Ovarian Immature Teratoma with Peritoneal Dissemination

Peritoneal dissemination of ovarian immature teratoma in children is a rare entity and is associated with a poor prognosis. There are no guidelines on the optimal management of these patients with regard to surgery and systemic treatments. This is the case of a 16-year-old patient who experienced an early recurrence of immature teratoma with peritoneal dissemination after surgery and systemic chemotherapy failure, and was treated with an aggressive salvage cytoreductive surgery. She was still disease-free 6 months after her second surgery. Upfront aggressive surgical management with complete cytoreductive surgery is recommended when patients present with disease recurrence and peritoneal dissemination of ovarian immature teratoma. Such cases should be managed in centers with local expertise in the treatment of peritoneal surface malignancies.

Dysgerminoma in a Prepubertal Girl with Complete 46XY Gonadal Dysgenesis: Case Report and Review of the Literature

Complete 46XY gonadal dysgenesis (Swyer syndrome) is a rare and challenging diagnosis among prepubertal girls, as estrogen insufficiency becomes evident only during adolescence, with nonspecific symptoms such as primary amenorrhea and/or delayed puberty. Unfortunately, girls with Swyer syndrome are at high risk for malignancies in the dysgenetic gonads, which can be prevented only by performing prophylactic bilateral gonadectomy. We present a 9-year-old patient with Swyer syndrome diagnosed with dysgerminoma in the right gonad and gonadoblastoma in the left gonad after prophylactic bilateral gonadectomy. Concerning the high risk of early gonadoblastoma and its malignant transformation, we recommend performing prophylactic bilateral gonadectomy at the time of diagnosis, even if the patient is prepubertal.

Aggressive B-Cell Lymphoma Involving the Appendix and Bilateral Ovaries in an 11-Year-Old Girl

The appendix and ovaries are rarely involved simultaneously in malignancies. The decision to perform an ovarian biopsy or a surgical resection in young patients can be challenging without sufficient clinical information. We describe an 11-year-old girl with bilateral ovarian masses, an enlarged appendix, associated pleural effusion, and ascites. Appendectomy and biopsy of the bilateral ovarian masses led to a diagnosis of aggressive B-cell non-Hodgkin lymphoma. The patient was treated with chemotherapy, which achieved complete remission and bilateral ovarian preservation. If ovarian involvement in malignant lymphoma is suspected, diagnostic methods should spare the ovary and prevent a loss of fertility. To evaluate for possible chemotherapy-induced ovarian damage, including infertility and premature menopause, an interdisciplinary approach is needed for the long-term follow-up of adolescent girls.

Prevention of Recurrent Mucinous Borderline Ovarian Tumor with Aromatase Inhibitor

Aromatase inhibitors (AIs) are used for estrogen-modulated conditions. Some borderline ovarian tumors (BOT) express estrogen receptors. We present 2 cases of progression from mucinous cystadenoma to mucinous BOT (mBOT) after prior cystectomies in whom an AI was used with recurrence prevention. Two patients underwent laparoscopic ovarian cystectomy for mucinous cystadenoma. Serial imaging demonstrated recurrent ovarian cysts for which both underwent fertility sparing surgery (FSS) with ovarian cystectomy for mBOT. Both patients were initiated on an AI and have been without recurrence. BOT predominantly occur in reproductive aged females. FSS with cystectomy is an option, but recurrence occurs in 12-36% of cases. The use of AI in prevention of recurrent BOT shows promise, and more studies are needed to explore this treatment.

The Role of Surgery in Antenatal Ovarian Torsion: Retrospective Evaluation of 28 Cases and Review of the Literature

Antenatal ovarian torsion (AOT) is rare and requires differentiating from other congenital cystic masses of the abdomen and pelvis in neonates. In this study, it was aimed to evaluate the prenatal characteristics and postnatal outcomes of AOT. DESIGN, SETTING, PARTICIPANTS, INTERVENTIONS, AND MAIN OUTCOME MEASURES: Hospital records of patients (n = 28) with diagnosis of AOT between 2004 and 2020 were reviewed and their prenatal characteristics, postnatal examination, imaging, operative, and histopathological findings were evaluated. There were 28 patients during the study period. In most of the patients (25/28; 89.3%) AOTs were detected prenatally. All were term babies with a mean birth weight of 3010 ± 466.6 g. Mean maternal age was 25.75 ± 3.65 years. Prenatal history was event-free in all and none had additional anomalies. Physical examination revealed mobile intra-abdominal cystic lesions in 16 (57.1%) patients. AOTs mimicked other pathologies as intestinal duplication cyst or mesenteric cyst (n = 7), complex ovarian cyst (n = 3), mature cystic teratoma (n = 3), simple renal cyst (n = 1), and ectopic kidney (n = 1) in 15 (53.6%) patients in postnatal ultrasonography. Elective laparoscopic surgery was performed in 26 (92.8%) patients. The other 2 patients required emergency open surgery because of acute symptoms. Ovaries were autoamputated in 17 (60.7%) patients. Histopathological examination revealed necrosis and dystrophic calcification in all specimens. There was neoplastic involvement in 2 patients (serous cystadenoma and gonadoblastoma). No complication occurred in the early follow-up period (mean: 73.9 ± 46.8 months). AOTs can be detected easily during the antenatal period. Neoplastic involvement with AOTs is rare but possible. Although AOTs might mimic other cystic pathologies, it should be one of the first diagnoses to be considered, in the presence of a palpable intra-abdominal mass and complex cystic lesion in infant girls. It can be efficiently and safely managed using minimally invasive techniques.

Growing Teratoma Syndrome and Gliomatosis Peritonei in a 15-Year-Old Girl With Immature Ovarian Teratoma: Case Report and Review of the Literature

Gliomatosis peritonei (GP) is a rare clinical condition characterized by presence of mature glial cells in the peritoneum. Growing teratoma syndrome (GTS) is described as an uncommon phenomenon that could be related to the incidence of non-seminomatous germ cell tumors. We report a case of a patient treated for immature ovarian teratoma, in whom both GP and GTS were observed, an association to date scarcely described in literature. A 15-year-old girl presented to the emergency department with severe pain in her lower abdomen and right lumbar region. Upon admission, concentration of α-fetoprotein (AFP) was 1500 ng/mL and β-human chorionic gonadotropin (β-hCG) less than 2 ng/mL. A computed tomography (CT) scan of the abdominal cavity and pelvis confirmed the presence of an anomalous mass in the abdominal cavity and pelvis. Initial surgery was performed. Histopathology revealed the presence of immature teratoma with epithelial elements. Normalization of AFP was achieved within 8 weeks. Five months after surgery, a progressive increase in AFP was noted. Magnetic resonance imaging (MRI) and CT scans of the pelvis minor showed local relapse. Evaluation of the remission after 2 blocks of preoperative chemotherapy revealed the presence of a large tumor mass in the pelvis minor, despite normalization of the AFP concentration. After opening the abdominal walls, numerous abnormal white nodules were observed in the peritoneum. Histopathology revealed the presence of mature glial tissue (gliomatosis peritonei). The remaining tumor mass was removed, and histopathology confirmed existence of mature teratoma (growing teratoma syndrome). Postoperative chemotherapy was continued. To date, the patient remains under clinical and laboratory remission. Concomitant incidence of GP and GTS, although rare, should always be taken into consideration in pediatric patients with diagnosis of either condition.

Two Concurrent Cancers in a 19-Year-Old Patient: Yolk Sac Ovarian Tumor and Metastatic Gastrointestinal Tract Malignancy—Case Report

Two concurrent malignancies present in a young person is an extremely rare condition. Diagnoses of gastric cancer primarily concern older patients. There are very few reports of concomitant Krukenberg tumor and germ-cell ovarian malignancy. A 19-year-old girl was admitted to the gynecologic oncology department with symptoms of advanced malignancy. Radiological imaging revealed disseminated neoplastic disease with bulky adnexal tumors. Cytoreductive surgery was performed, achieving no visible disease (T = 0 cm). The final pathology report confirmed metastatic mixed adenoneuroendocrine carcinoma (MANEC) in both ovaries, originating from the gastrointestinal tract. Moreover, the primary germ cell yolk sac tumor was found in the left ovary. In cases of concomitant gastric and ovarian tumors, metastatic disease (Krukenberg tumor) should be considered in the differential diagnosis. This concerns even adolescent patients. In particular cases, including tumors with germ cell components, primary debulking surgery is crucial for prognosis.

Analysis of Outcomes and Prognostic Factors after Fertility-Sparing Surgery in Patients with Early Stage Juvenile Granulosa Cell Tumor of the Ovary: Experience from a Tertiary Center

The objective of the study was to analyze the oncological outcomes and prognostic factors in patients with early-stage juvenile granulosa cell tumor (JGCT) who underwent fertility-sparing surgery. All patients with early-stage JGCT who underwent fertility-sparing surgery between January 1995 and December 2017 were reviewed retrospectively. None. The risk factors for recurrence and death in patients with early-stage JGCT were identified. Thirty-five patients were eligible for the current study. The median age was 17 years (range: 4-30 years), and 10 patients were premenarchal. Nine patients were International Federation of Obstetrics and Gynecology stage IA, and 26 were stage IC. Eight subjects underwent complete staging, whereas 28 had no staging at their initial surgery. In those without initial staging, 3 patients who received initial unilateral salpingo-oophorectomy (USO) and 6 with initial cystectomy underwent secondary surgery for staging or USO with staging, respectively. At the time of the secondary staging operation, 1 of the subjects (treated with USO at initial surgery) was found to be stage III and was excluded from this study. Therefore, 8 patients underwent secondary staging surgery performed by laparoscopic surgery, and none of them suffered recurrence during follow-up. Fourteen of the 16 patients with complete staging surgery underwent lymphadenectomy, and none of them had lymph node metastasis. Thirty-one patients received adjuvant chemotherapy after surgery. Eight patients had disease recurrence after a median follow-up time of 51 months (range: 6-229 months), with a median time to recurrence of 4.5 months (range: 2-52 months). Six patients died of their disease. The 5-year disease-free and overall survival rates were 74.8% and 84.3%, respectively. Univariate analysis showed that incomplete staging surgery was associated with increased risk of recurrence (P = 0.029). Adjuvant chemotherapy was not associated with disease-free survival. Four patients had a total of 6 pregnancies, resulting in 6 live births. Complete surgical staging is recommended for early-stage JGCT, but lymph node dissection can be omitted. Laparoscopic restaging surgery is feasible for patients with incomplete staging at initial surgery. However, the prognosis of patients with relapsed JGCT remains poor.

Progression of Cystadenoma to Mucinous Borderline Ovarian Tumor in Young Females: Case Series and Literature Review

To study the progression of benign ovarian lesions to mucinous borderline ovarian tumors (mBOTs); analyze the clinicopathologic features, diagnosis, and management of mBOTs in pediatric and adolescent girls; and provide a review of the literature on mBOTs in this population. Retrospective chart review of female adolescents younger than 18 years diagnosed with mBOTs between July 2017 and February 2021. Yale New Haven Hospital, New Haven, Connecticut; and Yale New Haven Health Bridgeport Hospital, Bridgeport, Connecticut. Three female patients diagnosed with mBOTs between ages 12 and 17 years. None. Clinical presentation, preoperative characteristics, surgical technique, histology, tumor stage, treatment, progression, outcome, and rate of recurrence. Three adolescent patients were identified to have mBOTs. All three patients presented with a chief complaint of abdominal pain. One of the 3 patients was premenarchal at presentation. Two of the 3 patients were initially diagnosed with a mucinous cystadenoma and had recurrences of an ovarian cyst in the same ovary within 5 and 17 months, respectively. Pathology of the recurrent cyst was consistent with mBOT. Two of the 3 patients initially underwent cystectomy, and all ultimately had a unilateral salpingo-oophorectomy. Subsequent surveillance over 2 to 4 years found no evidence of disease recurrence. mBOTs are rare in the pediatric and adolescent population and could arise from benign ovarian tumors.

Anti-N-Methyl-D-Aspartate Receptor Encephalitis Associated with Mature Ovarian Teratoma in a Young Adolescent: A Case Report

Ovarian teratoma has an uncommon association with anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis. This is a life-threatening condition, and here, we describe a case of an adolescent girl with anti-NMDAR encephalitis caused by an ovarian teratoma. A 14-year-old girl presented with acute features of fever, severe headache, altered behavioral changes, delirium, autonomic instability, episodes of seizure, and involuntary movement over a period of 1 month. Upon investigation, electroencephalogram (EEG), computed tomography (CT), and magnetic resonance imaging (MRI) of her brain showed normal findings. Her serum and cerebrospinal fluid were positive for anti-NMDARs, and a diagnosis of anti-NMDAR encephalitis was made, so she received a course of intravenous methyl prednisolone and immunoglobulin and was discharged after her neurological status improved. Upon further workup, she was suspected to have a left ovarian dermoid cyst on transabdominal ultrasonogram, which was an incidental finding. Her tumor marker panel showed normal serum lactate dehydrogenase, beta human chorionic gonadotropin, inhibin, alpha-fetoprotein, and carcinoembryonic antigen and a cancer antigen 125 level of 71.5 U/L. She eventually underwent laparoscopic left ovarian cystectomy and received immunotherapy in the postoperative period. She was discharged in stable condition on postoperative day 4. On histopathological examination, the specimen revealed a mature cystic teratoma with glial component. One year after surgery, the patient has recovered completely and has no residual psychiatric or neurological symptoms. Complete recovery after surgery in cases of anti-NMDAR encephalitis with ovarian teratoma emphasizes the need for early recognition of the entity, search for underlying tumor, and tumor removal to improve the prognosis.

Computed Tomography and Clinical Analysis of Ovarian Mucinous Tumors in Adolescent Patients

To investigate the clinical and computed tomography features of ovarian mucinous tumors in children and adolescents. A retrospective analysis of clinical and preoperative computed tomography (CT) data was performed in 59 patients who were 20 years or younger with ovarian mucinous tumors confirmed by histopathology. Patients' age, medical history, symptoms, tumor marker levels, and CT imaging findings were recorded. None. Identification of the clinical and CT features of ovarian mucinous tumors in pediatric and adolescent patients. There were 41 mucinous cystadenomas in 39 cases, 12 borderline mucinous cystadenomas in 11 cases, and 9 mucinous cystadenocarcinomas in 9 patients. A total of 55 tumors were multilocular (55/62, 88.7%), including fewer than 10 loculations in 23 tumors, 10-20 loculations in 17 tumors, and over 20 loculations in 15 tumors. Eleven borderline mucinous cystadenomas were multilocular (11/12, 91.7%), with over 10 loculations in 7 tumors (7/12, 58.3%). Twelve tumors appeared as multilocular cystic-solid (12/62, 19.4%), and a case of mucinous cystadenocarcinoma was predominantly solid (1/62, 1.6%). Seventeen tumors showed honeycomb sign and stained glass appearance. Six mural nodules, with sizes ranging from 1.1 to 3.5 cm (average: 2.8 cm), were found in borderline mucinous cystadenoma and mucinous cystadenocarcinoma. The CT findings of ovarian mucinous tumors in children are characteristic. The preoperative CT is helpful in making differential diagnoses.

Surgical Approach to Pediatric Ovarian Growing Teratoma Syndrome: A Case Report

Growing teratoma syndrome is defined as conversion of a metastatic immature tumor to a mature tumor after adjuvant chemotherapy and remains an area of investigation because of its unclear pathogenesis. Because of its risk of malignant transformation, the primary treatment strategy for pediatric patients is surgical resection. In this report we present a case of a pediatric patient with recurrent growing teratoma syndrome who was treated with chemotherapy, debulking procedures, and cryoablation for the growing nodules throughout her abdominal cavity. The patient has had a good clinical outcome without recurrent malignant tumor. These masses do not always regress with chemotherapy and complete surgical excision or ablation should be attempted when possible.

Interdisciplinary Management of Malignant Ovarian Tumors in the Pediatric and Adolescent Age Group

Malignant ovarian neoplasms are uncommon in the pediatric and adolescent population. Imaging and tumor markers help to guide the preoperative risk/benefit analysis for planned surgical management, which is the mainstay of therapy. An interdisciplinary approach should be taken in the management of this vulnerable population from diagnosis through post-treatment surveillance. In this review, the initial evaluation, risk stratification, and management of various types of malignant ovarian masses will be addressed, with a special focus on how to optimize an interdisciplinary approach to ovarian masses.

Multidisciplinary Clinical Considerations in the Treatment of Pediatric Epithelial Ovarian Cancer

Epithelial ovarian cancers are a rare subset of the less than 1% of ovarian cancers diagnosed in children. This case highlights considerations when caring for these patients. Evaluation of a 12-year-old postmenarchal girl who presented with suprapubic pain revealed a solid/cystic pelvic mass involving bilateral adnexa and elevated Cancer Antigen 125 (CA-125) level. Diagnostic laparoscopy pathology confirmed low-grade papillary serous ovarian carcinoma. Treatment involved surgical tumor debulking, hysterectomy, bilateral salpingo-oophorectomy, and omentectomy; adjuvant chemotherapy with no residual disease, and normalization of Cancer Antigen 125 (CA-125) level; and an aromatase inhibitor for maintenance. In children with adult-type gynecologic cancers necessitating treatments including surgical sterilization and hormone-modulating therapy, psychological support and developmentally informed collaboration between adult and pediatric services is essential. Clinical decisions for long-term bone and sexual health present opportunities for future research.

Swyer Syndrome: A Case of Dysgerminoma Solely within the Fallopian Tube

46XY pure gonadal dysgenesis (Swyer syndrome) is a rare disorder of sexual development. Patients have a 46XY karyotype, though phenotypically they appear female with normal external genitalia and vagina. Although patients exhibit normal Müllerian structures (uterus, fallopian tubes, and vagina), they possess a pair of bilateral undifferentiated gonad streaks. Delayed puberty and primary amenorrhea are the common presentations. There is an increased risk of developing tumors in the gonads and therefore a bilateral gonadectomy is recommended. A 16-year-old girl who presented with primary amenorrhea was diagnosed with Swyer syndrome. She underwent prophylactic bilateral gonadectomy and salpingectomies. She was discovered to have no gonadal malignancy, conversely dysgerminoma solely within the fallopian tube. Both bilateral salpingectomies and bilateral gonadectomies should be recommended as the operation of choice in patients with Swyer Syndrome.

Recommendations for Postoperative Surveillance of Pediatric Benign Ovarian Neoplasms

To assess postoperative management of pediatric patients with benign ovarian neoplasms, to develop recommendations for postoperative care. A retrospective cohort study. Eight pediatric hospitals in the midwestern United States. Patients up to 21 years of age who underwent surgery for a benign ovarian neoplasm between January 2010 and December 2016 were included. No prospective interventions were evaluated. Main outcome measures included postoperative imaging findings, recurrence rates, reoperation rates, and the timing of the aforementioned results. A total of 427 patients met inclusion criteria. After the index surgery, 155 patients (36%) underwent a routine imaging study. Among those with routine imaging, abnormalities were noted in 48 patients (31%); 7 went on to have reoperation (5%), and no malignant pathologies or torsion were identified. Excluding the 7 patients who went on to have a reoperation as a result of routine imaging, 113 patients developed symptoms postoperatively and underwent imaging as a result (27%, 113/420). Abnormalities were noted in 44 (10%); 15 of these patients underwent reoperation (4%), among them 2 with malignancies and 3 with torsion. Of these 44 patients, 23 had initially undergone routine imaging and subsequently went on to have symptomatic imaging, with 17% (4/23) undergoing reoperation. Routine imaging did not identify malignancy; most lesions identified on routine imaging were incidental findings. Although the study was not powered to appreciate a statistically significant difference, patients with malignancy or torsion were identified in the symptomatic group. This suggests no benefit from routine imaging, and supports symptomatic imaging postoperatively to minimize costs and patient/family burden.

Laparoscopic Trachelectomy for Cervix Yolk Sac Tumor in 11-Month-Old Girl: The Youngest Case

Yolk sac tumor (YST) is a malignant entity that often occurs in girls less than 3 years of age and is the most frequent type of primary extragonadal germ cell tumor. We describe the case of an 11-month-old girl who was referred to our center for vaginal bleeding with evidence of a uterine mass on ultrasonography. Preoperative investigations confirmed YST of the uterine cervix without metastasis. After 4 cycles of systemic chemotherapy, the patient was treated with laparoscopic trachelectomy (fertility-sparing surgery) without perioperative complications. After 12 months of follow-up, no residual mass was seen. The laparoscopic technique for trachelectomy for uterine cervix YST seems to be feasible and safe in children under 1 year of age.

Benign, Borderline, and Malignant Pediatric Adnexal Masses: A 10-Year Review

To investigate the incidence, clinical features, tumor markers, radiologic findings, types of surgeries, and histologies for adnexal masses in female pediatric and adolescent patients. Retrospective chart review. Children's Health in Dallas and Plano, Texas from 2009 to 2018. Female patients younger than 19 years old who underwent surgical management of an adnexal mass. None. Imaging characteristics, tumor markers, surgical procedures, and histopathology. In total, 752 patients (mean age, 13.7 years) underwent 756 surgical procedures for 781 adnexal masses. Of these, 732/781 (93.7%) were benign, 7/781 (0.9%) were borderline, and 42/781 (5.4%) were malignant. Of all 781 masses, 520/781 (66.6%) were ovarian and 261/781 (33.4%) were paratubal or tubal. Benign masses were associated with Hispanic race, pain, simple or cystic characteristics on imaging, and negative tumor markers. Borderline and malignant masses were associated with white race, pain, mass or distension, larger size, and heterogeneous appearance on imaging. Borderline masses were associated with negative tumor markers. Malignant masses were associated with elevated alpha fetoprotein, beta human chorionic gonadotropin, cancer antigen 125, and lactate dehydrogenase. Most adnexal masses in the pediatric and adolescent population are benign. Benign masses were significantly smaller, more likely to have negative tumor markers, and appear simple or cystic. There is little standardization with respect to preoperative tumor markers for adnexal masses. High-yield tumor markers for malignancy include alpha fetoprotein, beta human chorionic gonadotropin, cancer antigen 125, and lactate dehydrogenase. Low-yield tumor markers include inhibin A and B. Gynecologists performed more fertility-preserving surgeries including mini-laparotomies and fewer laparotomies for benign masses than pediatric surgeons.

DICER1 Mutation in Recurrent Ovarian Sertoli-Leydig Cell Tumor: A Case Report

DICER1 mutation has been linked to development of Sertoli-Leydig cell tumor and cystic nephroma, among other neoplasms. We present a unique case of recurrent ovarian Sertoli-Leydig cell tumor in a pediatric patient with a known DICER1 mutation and history of cystic nephroma. She underwent surgical staging and adjuvant chemotherapy, and her recurrences have been treated with chemotherapy, whole-abdomen radiation therapy, and further surgical debulking. This report adds to the small body of evidence about this rare but unexpectedly highly aggressive tumor, especially in the recurrent setting, and reminds the reader of the importance of cancer diagnosis in this population.

National Trends in Cervical Cancer Screening in Adolescents

To characterize patterns of cervical cancer screening among adolescents ages 14-20 years before and after the 2009 American College of Obstetrics and Gynecology recommendations. Retrospective cohort study. National Ambulatory Medical Care Survey 2005-2016 data. Female adolescents and young women ages 14-26 years. Multivariable logistic regression models identified independent predictors of unindicated cervical cancer screening at office-based visits among adolescents ages 14-20 years. Cervical cancer screening in women ages 14-20 and 21-26 years in 2 time periods: 2005-2008 and 2009-2016. Between 2005 and 2016, 11,768 office visits were among adolescents ages 14-20 years. Overall, the cervical cancer screening rate for young women ages 14-20 years was 4.0%, which decreased from 4.5% to 0.4% (P = .008) during the study period. Adolescents who received cervical cancer screening during an office visit were older (18-20 years: 24.1% vs 14-17 years: 8.2%; P < .001), had a preventive care visit (preventive care: 79.7% vs other visit types: 20.3%; P < .001), and saw an obstetrician/gynecologist (obstetrician/gynecologist: 74.81% vs other specialties: 25.1%; P < .001). After adjusting for age, year, period, insurance status, region, and provider type, screening for cervical cancer was associated with living in the Southern region of the United States (adjusted odds ratio, 1.88; 95% confidence interval, 1.09-3.25; P = .02) and public insurance (adjusted odds ratio, 0.47, 95% confidence interval, 0.34-0.64; P < .001). Despite recommendations, cervical cancer screening continued to occur in the adolescent population-especially older adolescents-creating unnecessary costs and potential harms. These findings show a slow uptake of guidelines nationally and the need for initiatives that encourage implementation of performance measures for providers, including ongoing provider and patient education.

Diagnostic Performance of Magnetic Resonance Imaging for Pediatric Ovarian Neoplasms: A Multi-Institutional Review

To assess the diagnostic performance of MRI to predict ovarian malignancy alone and compared with other diagnostic studies. A retrospective analysis was conducted of patients aged 2-21 years who underwent ovarian mass resection between 2009 and 2021 at 11 pediatric hospitals. Sociodemographic information, clinical and imaging findings, tumor markers, and operative and pathology details were collected. Diagnostic performance for detecting malignancy was assessed by calculating sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) for MRI with other diagnostic modalities. One thousand and fifty-three patients, with a median age of 14.6 years, underwent resection of an ovarian mass; 10% (110/1053) had malignant disease on pathology, and 13% (136/1053) underwent preoperative MRI. MRI sensitivity, specificity, PPV, and NPV were 60%, 94%, 60%, and 94%. Ultrasound sensitivity, specificity, PPV, and NPV were 31%, 99%, 73%, and 95%. Tumor marker sensitivity, specificity, PPV, and NPV were 90%, 46%, 22%, and 96%. MRI and ultrasound concordance was 88%, with sensitivity, specificity, PPV, and NPV of 33%, 99%, 75%, and 94%. MRI sensitivity in ultrasound-discordant cases was 100%. MRI and tumor marker concordance was 88% with sensitivity, specificity, PPV, and NPV of 100%, 86%, 64%, and 100%. MRI specificity in tumor marker-discordant cases was 100%. Diagnostic modalities used to assess ovarian neoplasms in pediatric patients typically agree. In cases of disagreement, MRI is more sensitive for malignancy than ultrasound and more specific than tumor markers. Selective use of MRI with preoperative ultrasound and tumor markers may be beneficial when the risk of malignancy is uncertain. This retrospective review of 1053 patients aged 2-21 years who underwent ovarian mass resection between 2009 and 2021 at 11 pediatric hospitals found that ultrasound, tumor markers, and MRI tend to agree on benign vs malignant, but in cases of disagreement, MRI is more sensitive for malignancy than ultrasound.

Nongestational Ovarian Choriocarcinoma with Precocious Puberty in a 7-Year-Old Girl: A Rare Case Report

Nongestational ovarian choriocarcinoma (NGOC) is extremely rare, particularly in the pediatric population. Few case reports have discussed NGOC with precocious puberty and mentioned both tumor markers (β-human chorionic gonadotropin [hCG], alpha-fetoprotein, CA125) and the dynamics of gonadotropic and gonadal hormones. A 7-year-old girl presented with early puberty and an abdominal mass. Laboratory tests revealed elevated estradiol, β-hCG, and CA125 levels, with suppressed luteinizing hormone and follicle-stimulating hormone. She was diagnosed with ovarian choriocarcinoma and precocious puberty. Following tumor resection, her hormone levels normalized, and symptoms resolved. Pediatric ovarian choriocarcinomas are rare and may present with precocious puberty. Comprehensive endocrine evaluation, including serum hormone assays, is essential for diagnosis. Hormonal profiles in pediatric patients may differ from those in adults, with estradiol levels often being markedly elevated compared to age-specific reference ranges, and progesterone levels being relatively low.

Isolated Inhibin-Producing Granulosa Cell Tumor Causing Primary Amenorrhea: A Case Report

Juvenile granulosa cell tumors (JGCTs) are the most common pediatric ovarian sex cord-stromal tumor. Because most granulosa cell tumors are hormonally active, patients commonly present with signs and symptoms of endocrine dysfunction, including precocious puberty and menstrual irregularities. Most patients with JGCTs present with stage I disease confined to the ovary that can be managed with surgical excision alone with a favorable prognosis. A 15-year-old girl presented with primary amenorrhea. Evaluation revealed suppressed follicle-stimulating hormone, elevated luteinizing hormone, normal estradiol, and a markedly elevated inhibin B, and pelvic ultrasound and magnetic resonance imaging showed a mildly enlarged right ovary with a 1.6-cm "ovarian follicle." After consultation with gynecologic oncology, the patient underwent right salpingo-oophorectomy with histopathologic findings consistent with JGCT. Menarche subsequently occurred 2 months after surgery. Differences in GCT endocrine function, as well as the stage of pubertal development when the tumor develops, may lead to a variety of clinical presentations. It is important to consider inhibin-producing JGCT on the differential diagnosis for pediatric and adolescent patients presenting with amenorrhea.

Giant Mitotically Active Cellular Fibroma of the Ovary in an Adolescent

Mitotically active cellular fibroma (MACF) of the ovary is a rare stromal tumor, representing an intermediate entity between cellular fibromas and fibrosarcomas. While fibrosarcomas show aggressive clinical behavior with nuclear atypia and high mitotic counts, MACFs demonstrate increased mitotic activity without atypia. Pediatric cases are exceptionally uncommon. A 15-year-old adolescent presented with an adnexal mass. Surgical excision revealed a large ovarian tumor, confirmed histopathologically as a mitotically active cellular fibroma. The tumor was the largest and heaviest MACF reported in a pediatric patient to date. The postoperative course was uneventful, and the patient remains under follow-up without recurrence. Ovarian MACF is a rare diagnosis in children and adolescents. Its recognition is important, as it carries intermediate malignant potential and may mimic fibrosarcoma. Accurate histopathological evaluation is essential to guide management and prevent overtreatment, while ensuring appropriate follow-up for early detection of recurrence.

Pubertal Development as a Marker of Gonadal Neoplasm in 46XY Complete Gonadal Dysgenesis

46XY Complete Gonadal Dysgenesis (CGD), also known as Swyer syndrome, is characterized by nonfunctional streak gonads and an increased risk of gonadal neoplasms. While spontaneous puberty is not expected, hormonally active gonadal tumors can mimic normal puberty delaying diagnosis. We present two adolescents with 46XY CGD and tumor-driven puberty: a 16-year-old with spontaneous thelarche and adrenarche found to have a left gonadoblastoma, and a 14-year-old with spontaneous menarche and pelvic pain who was subsequently diagnosed with a dysgerminoma and a sex cord tumor with annular tubules (SCTAT). Puberty in patients with 46XY CGD should prompt suspicion for gonadal tumors. Early recognition and gonadectomy are crucial to prevent malignant transformation and optimize outcomes.

Virilization, Ovarian Hyperthecosis, and Torsion Masquerading as Malignancy: A Case Report

Premenopausal females with signs of androgen excess and oligomenorrhea are commonly evaluated for polycystic ovarian syndrome or for an androgen-secreting tumor if a pelvic mass is present. Ovarian hyperthecosis (OHT) as a cause of these symptoms in adolescents is rare. A 15 year-old female with primary amenorrhea and signs of virilization was referred to Pediatric and Adolescent Gynecology after pelvic imaging demonstrated a pelvic mass, suspected to be arising from the right ovary, and an adjacent paratubal cyst. Preoperative laboratory evaluation revealed markedly elevated testosterone levels (free testosterone 81.5 pg/mL [normal <7.5 pg/mL]; total testosterone 279.6 ng/mL [normal <52.0 ng/mL]). Ovarian tumor markers were otherwise normal. Surgical evaluation revealed bilaterally enlarged ovaries and right paratubal cyst with right ovarian torsion. Rather than demonstrating an androgen-secreting tumor, pathologic evaluation following right salpingo-oophorectomy resulted in an unexpected diagnosis of OHT. Our case demonstrates that OHT should be considered in the differential in the setting of a large pelvic mass and virilization in an adolescent.

Gonadal Tumors in Individuals with Turner Syndrome and Y-Chromosome Mosaicism: A Retrospective Multisite Study

To evaluate the prevalence of germ cell tumors and the clinical monitoring practices for those who deferred prophylactic gonadectomy in a large North American cohort of individuals with Turner syndrome with Y-chromosome mosaicism (TS+Y). A query of the medical records at multiple North American children's hospitals was done using ICD codes related to Turner Syndrome. A retrospective chart review was conducted on those patients between ages 0 to 30 years with Y-mosaicism. The data of 57 participants were analyzed. Eight (25.8%, n = 31) ≥ 13 years underwent spontaneous thelarche. One (3.2%) had spontaneous menarche. Forty-seven (82.5%) had gonadectomy at a median age of 8 years (IQR 11.0, range <1 to 19 years). Sixteen (34%) had growth hormone therapy exposure prior to gonadectomy. Fourteen (29.8%) had gonadoblastoma. Two (4.3%) had dysgerminoma. Differences in age at gonadectomy, presence of the entire Y-chromosome, and exposure to growth hormone when comparing those with vs without gonadal tumor were not statistically significant. Gonadectomy had not been performed in 10 individuals, median age 6.5 (IQR 9.0, range <1 to 14 years). There was no consistency in the plan for ultrasound and/or tumor markers for follow-up. Our data shows a prevalence of 24.6% of gonadal tumors in individuals with TS +Y and a relatively low risk of malignant transformation (3.5%). Prior exposure to growth hormone was not predictive of the presence of gonadal tumor. Future cytogenetic studies are needed to better understand the factors involved in the development of gonadal tumors.

A Rare Ovarian Mixed Sex Cord Stromal Tumor in a Patient with Ollier Disease: A Case Report

This is a case report of a 10-year-old with Ollier disease and an ovarian mass. Ollier disease, a rare disorder characterized by multiple enchondromas resulting in bone deformities, has been occasionally associated with ovarian juvenile granulosa cell tumor. This patient developed signs of precocious puberty and was found to have an ovarian tumor; however, pathology revealed a mixed sex-cord stromal tumor with components of juvenile granulosa and Sertoli-Leydig cell tumor. Tumor genomic testing revealed an IDH1 mutation. Mixed sex-cord stromal tumors of this type, also called "gynandroblastomas," have been associated with DICER1 mutations and DICER1 tumor predisposition syndrome but never with Ollier disease. Our findings expand the known spectrum of syndromic associations with this tumor type, with implications for tumor screening.

Ovarian Dermoid Cyst Trajectory in Premenarchal Girls

Mature ovarian dermoid cysts (ODCs) are the most common benign ovarian tumors diagnosed in children. However, there is minimal data on management of ODCs in premenarchal patients. This study assesses characteristics associated with expectant (EM) vs surgical (SM) management in premenarchal patients and the growth rate of ODCs in EM patients at a single institution. Forty-four premenarchal patients, either post-surgical with pathologically-confirmed ODC or having radiologic findings consistent with ODCs, were included. Data collected included demographics, cyst characteristics, imaging findings, presence of symptoms, surgical procedure performed, and ovarian torsion occurrence. Patient age at diagnosis was similar between groups (SM: 8.8 vs EM: 8.0, P = .55). At presentation, 36 patients (82%) underwent SM and 8 (18%) underwent EM. There was a significant difference in cyst size between groups (SM: 8.9 cm vs EM: 3.6 cm, P = .004). Of SM patients, 30% underwent oophorectomy vs cystectomy, with a significant difference in ODC size between procedures (11.8 cm vs 7.7 cm, P = .016). Of EM patients, 75% had at least one and 60% had three follow-up ultrasounds, with average follow-up timeframes of 3.7 and 27 months respectively. Average yearly ODC growth rate for the latter group was 0.8 cm. The average yearly growth rate of ODCs in premenarchal patients within our institution was slower than in older cohorts, and both age and cyst size played significant roles in determining surgical procedure. Continued study on EM in premenarchal ODCs will help define parameters for recommending SM vs EM in this population.

Clinical and Computed Tomography Features of Female Pelvic Malignant Germ Cell Tumors in Children and Adolescents: A Series of 30 Cases

To investigate the clinical features and computed tomography (CT) findings of malignant pelvic germ cell tumors in female patients and improve the diagnostic level of this disease. The clinical and CT data of 30 female patients with malignant pelvic germ cell tumors confirmed using histopathology were retrospectively analyzed, before surgery, at a tertiary hospital. Patient's age, tumor location, clinical symptoms, tumor marker levels, and CT findings were recorded. None. Identification of the clinical features and CT findings of malignant pelvic germ cell tumors. The patients ranged in age from 11 months to 22 years old, with a mean age of 12.3 years. The 30 cases (30 tumors) included 10 cases of yolk sac tumor, 8 cases of dysgerminoma, and 5 cases of immature teratoma. The alpha-fetoprotein levels were elevated in 8 cases of yolk sac tumor. Eight cases of dysgerminoma showed lobulation sign. Four cases of immature teratoma contained fat and calcification. The fibrovascular septa were observed on contrast-enhanced CT images in 4 cases of dysgerminoma. The "bright dot sign" was noted on contrast enhanced CT images in 9 cases of yolk sac tumor. Remarkable enhancement of the solid component or septa was detected in 10 cases of yolk sac tumor. Obvious enhancement surrounding vascular bundles in the tumor was observed in 5 cases of dysgerminoma. Malignant pelvic germ cell tumors in female patients frequently affect children and adolescents. The CT findings of dysgerminoma, immature teratoma, and yolk sac tumor have certain characteristic features. The combination of CT manifestations, tumor markers, and patient age helps improve the diagnostic level of this disease.

Human Papilloma Virus Vaccination Among Female Patients Attending French Pediatric Cystic Fibrosis Centers

To describe human papilloma virus (HPV) vaccination practice among adolescent girls with cystic fibrosis (CF) and to identify reasons for non-vaccination. Cross-sectional multicentric study. Girls aged 9-17 years, attending 7 French pediatric CF centers, and their accompanying adult. Administration of a self-report questionnaire. The proportion of girls having received or receiving HPV vaccination, compliance with the vaccination schedule, factors associated with vaccination, and reasons for vaccination and for non-vaccination. A total of 113 girls and 104 accompanying adults participated. The mean age was 13.6 years (standard deviation 2.5; range 9-17). A total of 34 (30.9%) patients reported having received HPV vaccination. Among the 34 girls aged 15 years or older, 15 (44.1%) were vaccinated. Most patients (58.8%) started vaccination between 11 and 14 years of age (mean age 13.9). Most vaccine prescriptions (67.6%) were made by a CF center health care provider. Factors associated with vaccination were older age (odds ratio [OR] = 1.27, 95% confidence interval [CI] = 1.01-1.6, P = .037 for each year older), previous vaccination by the accompanying parent of one of their children for hepatitis B (OR = 8.01, 95% CI = 0.96-67.02), P = .055), and parental influence on decision-making (OR = 2.77, 95% CI = 0.97-7.95, P = .058). Health care providers' positive advice and fear of HPV-related disease were the main reasons given to justify vaccination decisions. Insufficient knowledge and concerns about potential side effects were the main barriers. HPV vaccination remains insufficient among girls with CF. CF health care providers may play a crucial role in HPV vaccination acceptance, and their sensitization to cervical cancer prevention is mandatory.

Vaginal Metastases of Wilms’ Tumor in a Pediatric Patient: A Rare Case

Wilms' tumor is the second most common pediatric abdominal cancer; however, it rarely involves the female reproductive tract. There are few cases reported in the literature describing uterine, ovarian, cervical, and vaginal involvement. We report the case of a 7-year-old girl presenting with a large renal mass with retroperitoneal nodal and lung metastases; she was diagnosed with stage 4 favorable histology Wilms' tumor. She was treated with surgery, chemotherapy, and radiation. She presented with vaginal bleeding 10 months after completing treatment; biopsy of a vaginal mass confirmed recurrence, and this was sent for molecular profiling, which did not identify an inherited cancer predisposition or targetable mutation. She was again treated with chemotherapy; examination redemonstrated a small vaginal mass, but re-biopsy of the lesion was negative for malignancy. Due to high risk of local relapse, ongoing chemotherapy and pelvic radiation ensued. End-of-treatment imaging and vaginoscopy showed no residual disease. Vaginal metastases of Wilms' tumor are very rare; this is the second reported case in the literature. Pediatric clinicians should have a strong suspicion for vaginal metastases in cancer patients presenting with vaginal bleeding, especially when their pubertal development does not suggest that bleeding would be secondary to menarche. Long-term gynecologic care for these patients is paramount to reduce morbidity from chemotherapy and pelvic radiation. Fertility preservation counselling should be made early, through referral to a specialist.

Single-Dose Human Papillomavirus Vaccination in Low- and Middle-Income Countries—Time for Implementation?

Although cervical cancer is becoming a rare disease in high income regions, it is still a major health issue in low- and middle-income countries (LMICs). Cervical cancer develops after infection with a high-risk human papilloma virus (hrHPV), an infection against which vaccination has been possible since 2006. Large population immunization programs have been organized in many higher income countries, and yet they have not been implemented in most of the lower and middle-income nations. The cost of the vaccine, as well as the need for two doses impedes coverage in the most vulnerable groups. Studies are suggesting the efficacy of single dose vaccination, but so far only observational data are available while large, randomized, double-blind studies are still ongoing. In order to prevent and combat this disease, it is essential to inform the population of vaccination benefits and offer accessible programs in higher as well as low-and middle-income countries. In this commentary, we wish to focus our attention on the case for implementing single dose vaccination in lower- and middle-income nations.

Rare Pediatric Vaginal Granular Cell Tumor: A Case Report

Granular cell tumors (GCTs) are usually benign and occur primarily in the trunk and head. Vaginal tumors are rare, particularly in children. A 9-year-old girl was admitted with irregular vaginal bleeding for 1 year. The bleeding had worsened in the previous 1 month. B-ultrasound and computed tomography showed a round mass in the vagina. We performed vaginoscopy using a hysteroscopic exploration and tumor resection via an open technique. The pathology of the resected tumor confirmed a vaginal wall GCT. No recurrence was noted in the following 16 months. To our knowledge, our prepubescent girl is the youngest patient with GCT in the literature. Histopathological evaluation and complete tumor resection with clear margins is the primary treatment for benign or malignant GCTs.

Prepubertal Vaginal Bleeding: An Inpatient Series from a Single Center in Fujian China

This study characterizes the etiology, clinical features and outcomes of prepubescent vaginal bleeding and summarizes our experience. The data of patients who were hospitalized with vaginal discharge or bleeding treated from January 2012 to December 2018 were retrospectively reviewed. A provincial Grade III Level A Maternity and Children's Hospital. Prepubertal patients with vaginal discharge or bleeding. Patient age, bleeding duration, etiology, treatment and prognosis were recorded. Physical examinations and color Doppler ultrasonography were also performed. There were 158 patients aged from 1 month to 10 years (mean age 5.2 years). Bleeding duration ranged from 1 to 98 days, with an average of 13.3 days. Sixty patients were diagnosed with vaginal foreign bodies, 34 with vulvovaginitis, 34 with vulvar trauma, 13 with ovarian granulosa cell tumors, 8 with urethral mucosa prolapse, 5 with vaginal yolk sac tumors and 1 each with pituitary tumor, hypothyroidism, McCune-Albright syndrome, and short-term intake of a large number of strawberries. All the children were treated according to their different disease etiologies. Prepubertal vaginal bleeding is caused by a variety of different conditions. In our study, the most common causes were vaginal foreign bodies, vulvovaginitis, trauma, vaginal malignant tumors and urethral mucosa prolapse. Careful medical histories and targeted examinations are needed. Vaginoscopy could be considered. Considering the different causes, different treatments should be administered to achieve a good prognosis.

Ovarian Torsion in Adolescents with and without ovarian mass: A Cross-sectional Study

The aim of this study was to compare the characteristics of adolescents with and without adnexal mass who were diagnosed with ovarian torsion (OT). Retrospective cross-sectional study. Gynecology Department at Tepecik Education and Reseach Hospital, Izmir, Turkey. Adolescent girls who received surgery for OT between March 2012 and October 2020 in our institution. The patients were divided into 2 groups according to the presence or absence of an ovarian mass and compared. Demographic and clinical characteristics, imaging findings, surgery, and pathology reports of the patients were obtained. Differences in initial symptoms, ultrasound findings, the diagnostic process, and the degree of torsion in patients with and without ovarian mass. Seventy-six patients were diagnosed with OT. Of the 76 patients, 41/76 (53.9%) had an ovarian mass (OTwM), and 35/76 (46.1%) had no pathology (OTnP). The admission to surgery interval was longer in the OTnP group (P = .03). Ultrasound findings of ovarian edema and the appearance of free fluid were significant in the OTnP group (P = .001). The largest dimension of the nontorsion ovary in the OTnP group was greater than in the OTwM group (P = .03). In addition, it was found that torsion more than 360° was more common in the OTnP group than in the OTwM group (24/35, 68.5% vs 41/18, 43.9%; P = .03). In symptomatic adolescents without an ovarian mass, the presence of follicle peripheralization suggesting ovarian edema on ultrasound and the presence of free fluid in the abdomen are important in terms of suspicion of torsion for clinicians. In addition, the degree of torsion was increased in these patients.

Use and Accuracy of Intraoperative Frozen Section Analysis for Ovarian Masses in Children and Adolescents

Describe the current practice patterns and diagnostic accuracy of frozen section (FS) pathology for children and adolescents with ovarian masses DESIGN: Prospective cohort study from 2018 to 2021 SETTING: Eleven children's hospitals PARTICIPANTS: Females age 6-21 years undergoing surgical management of an ovarian mass INTERVENTIONS: Obtaining intraoperative FS pathology MAIN OUTCOME MEASURE: Diagnostic accuracy of FS pathology RESULTS: Of 691 patients who underwent surgical management of an ovarian mass, FS was performed in 27 (3.9%), of which 9 (33.3%) had a final malignant pathology. Among FS patients, 12 of 27 (44.4%) underwent ovary-sparing surgery, and 15 of 27 (55.5%) underwent oophorectomy with or without other procedures. FS results were disparate from final pathology in 7 of 27 (25.9%) cases. FS had a sensitivity of 44.4% and specificity of 94.4% for identifying malignancy, with a c-statistic of 0.69. Malignant diagnoses missed on FS included serous borderline tumor (n = 1), mucinous borderline tumor (n = 2), mucinous carcinoma (n = 1), and immature teratoma (n = 1). FS did not guide intervention in 10 of 27 (37.0%) patients: 9 with benign FS underwent oophorectomy, and 1 with malignant FS did not undergo oophorectomy. Of the 9 patients who underwent oophorectomy with benign FS, 5 (55.6%) had benign and 4 (44.4%) had malignant final pathology. FSs are infrequently utilized for pediatric and adolescent ovarian masses and could be inaccurate for predicting malignancy and guiding operative decision-making. We recommend continued assessment and refinement of guidance before any standardization of use of FS to assist with intraoperative decision-making for surgical resection and staging in children and adolescents with ovarian masses.

Small Cell Carcinoma of Ovary, Hypercalcemic Type: A Rare Case Report

Small cell carcinoma of the ovary, hypercalcemic type (SCCOHT), is a rare neoplasm that occurs mostly in women younger than 40 years of age. The unknown histogenesis makes the diagnosis of SCCOHT difficult. SCCOHT was recently shown to be associated with SMARCA4 gene mutation. Serum calcium levels can be used as a marker of treatment response and relapse in SCCOHT. Here we report on a 16-year-old girl who presented with pelvic mass and hypercalcemia. SCCOHT was diagnosed histopathologically. However, loss of neither BRG1 nor INI1 expression was detected. The diagnosis of SCCOHT should be among the differential diagnoses in adolescents presenting with pelvic mass and hypercalcemia. We would like to share our experience with this rare case, discuss recent management, and emphasize the importance of a multidisciplinary approach. Although it is known that almost all cases have a loss of BRG1 expression and a small group exhibit loss of INI1, our patient indicates that there could be exceptional cases with hitherto undescribed genetic abnormalities.

Leiomyoma of the Vulva: Case Report

Leiomyoma of the vulva is rare, accounting for only 0.03% of all gynecological tumors, and it is seldom seen in teenagers. We describe a case of vulvar leiomyoma in a 14-year-old girl who presented a 10 × 10 cm solid tumor in the right hemivulva without other complaints. Differential diagnosis includes Bartholin cysts, abscesses, fibromas, and other solid lesions. Although rare, vaginal leiomyoma must be remembered and included as a differential diagnosis for solid lesions in the vagina.

Failure of Operative Therapy after Ovarian-Sparing Surgery for Pediatric Benign Ovarian Neoplasms: A Retrospective Review

To evaluate failure of initial operative therapy (incomplete tumor removal) of ovarian-sparing surgery for pediatric benign ovarian neoplasms. A retrospective review of patients up to 21 years of age who underwent ovarian-sparing surgery for a benign ovarian neoplasm from 2010 to 2016 at 8 pediatric hospitals was conducted. Failure of initial operative therapy is defined as a radiologically suspected or pathologically confirmed ipsilateral lesion with the same pathology as the primary neoplasm within 12 weeks of the initial operation. Forty patients received imaging within 12 weeks of their primary operation. Sixteen (40%) patients had a radiologically identified ovarian abnormality ipsilateral to the primary lesion, and 5 patients were suspected to have the same lesion as their primary neoplasm. Three of the 5 patients (7.5%) underwent reoperation with pathologic confirmation of the same lesion, resulting in a pathologically confirmed failure of therapy rate of 7.5%. The other 2 patients had serial imaging that subsequently demonstrated no recurrence with lesion resolution. Age, race/ethnicity, laparoscopy vs laparotomy, presence of torsion, pathology, size of lesion, and surgeon specialty were not associated with failure of therapy. In most patients who received imaging within 12 weeks of the primary operation for resection of a benign ovarian neoplasm, ovarian-sparing surgery was successful in complete tumor removal, with a low failure of therapy rate. Selected patients with suspected failure of therapy on initial imaging could be serially monitored to determine the need for repeat surgical intervention.

Vaginal Yolk Sac Tumor: A Case Series and Review of the Literature

To report diagnosis, treatment, and outcomes of vaginal yolk sac tumor (YST) cases at a single institution and review literature on vaginal YST to outline advancements in diagnosis, treatment, and survival. Retrospective chart review of female patients less than 21 years of age with pathologic diagnosis of vaginal YST treated at a large children's hospital, and summary of a 100-year review of the literature on vaginal yolk sac tumor. Children's Healthcare of Atlanta, a tertiary center in Atlanta, GA. Female patients less than 21 years of age diagnosed with vaginal YST. Two cases of vaginal YST at our institution are outlined. Both patients presented within the first 2 years of life with vaginal bleeding and were treated successfully with chemotherapy alone. After review of the literature, 137 cases of vaginal YST were found. The mean age at diagnosis was 11 months, and all patients presented with vaginal bleeding. Before 2000, more radical treatments were pursued, and 40% resulted in death. Since the year 2000, treatment has shifted toward chemotherapy and more conservative surgical management, with 51% of vaginal YST cases treated with chemotherapy alone with 92% of patients alive at time of publication. Our cases contribute to the limited literature demonstrating the efficacy of conservative management of rare cases of vaginal YST with chemotherapy alone. This case series and review of the literature provide mounting evidence that vaginal YST should be in the differential diagnosis in young girls with vaginal tumors, and conservative management of vaginal YST has excellent outcomes.

Early diagnosis and treatment improve outcomes for premenarchal patients with anti-NMDA receptor encephalitis and ovarian teratoma

Gonadectomy in Individuals with Turner Syndrome and Y Chromosome Material: Fertility Considerations

Minimally Invasive Ovarian-Preserving Approach for the Management of a Sclerosing Stromal Tumor in an Adolescent: A Case Report

Sclerosing stromal tumors (SSTs) are rare benign ovarian tumors that occur in adolescents and young adults. They are often treated with unilateral salpingo-oopherectomy due to concern for malignancy. A 13-year-old postpubertal female presented with sharp, constant abdominal pain with physical exam concerning for a lower abdominal mass. An ultrasound revealed a 9.7-cm solid, heterogenous left ovarian mass. The abdomen and pelvis CT confirmed the findings and showed a predominantly cystic mass arising from the left adnexa. During surgery, a smooth and distinct mass arising from the left ovary was encountered without abnormal findings in surrounding structures. A cystectomy was performed, and intraoperative findings showed no evidence of malignancy. Based on the lab, imaging, and surgical findings, staging and salpingo-oopherectomy were not pursued. Given the benign nature of SSTs, it is important to entertain the diagnosis in adolescents presenting with clinically congruent ovarian masses. The minimally invasive approach allows for sparing of the ovary in the adolescent population.

Multi-Institutional Review of the Preoperative Diagnostic Accuracy for Pediatric Ovarian Mature Cystic Teratomas

To assess the preoperative imaging impression and surgeon diagnostic accuracy for pediatric ovarian mature cystic teratomas (MCTs) DESIGN: Retrospective review SETTING: Eleven pediatric hospitals PARTICIPANTS: Patients ages 2 to 21 who underwent surgical management of an ovarian neoplasm or adnexal torsion with an associated ovarian lesion INTERVENTION: None MAIN OUTCOME MEASURES: Preoperative imaging impression, surgeon diagnosis, tumor markers, and pathology RESULTS: Our cohort included 946 females. Final pathology identified 422 (45%) MCTs, 405 (43%) other benign pathologies, and 119 (12%) malignancies. Preoperative imaging impression for MCTs had a 70% sensitivity, 92% specificity, 88% positive predictive value (PPV), and 79% negative predictive value (NPV). For the preoperative surgeon diagnosis, sensitivity was 59%, specificity 96%, PPV 92%, and NPV 74%. Some measures of diagnostic accuracy were affected by the presence of torsion, size of the lesion on imaging, imaging modality, and surgeon specialty. Of the 352 masses preoperatively thought to be MCTs, 14 were malignancies (4%). Eleven patients with inaccurately diagnosed malignancies had tumor markers evaluated and 82% had at least 1 elevated tumor marker, compared with 49% of those with MCTs. Diagnostic accuracy for the preoperative imaging impression and surgeon diagnosis is lower than expected for pediatric ovarian MCTs. For all ovarian neoplasms, preoperative risk assessment including a panel of tumor markers and a multidisciplinary review is recommended. This process could minimize the risk of misdiagnosis and improve operative planning to maximize the use of ovarian-sparing surgery for benign lesions and allow for appropriate resection and staging for lesions suspected to be malignant.

Adnexal Torsion Due to Borderline Mucinous Tumor of the Gonad in a Prepubertal Girl with Mixed Gonadal Dysgenesis (45,X/46,XY) and a Turner Phenotype

Turner syndrome (TS) is a sex chromosome condition characterized by complete or partial loss of the X chromosome. Patients with mixed gonadal dysgenesis (45,X/46,XY) and a Turner phenotype are predisposed to gonadoblastoma with malignant transformation. We present the case of a TS patient with 45,X/46,XY with 2 episodes of left adnexal torsion (AT). Biopsies during detorsion showed benign mucinous cystadenoma. Pathology following bilateral gonadectomy revealed a left gonad with mucinous borderline tumor and right gonad with gonadoblastoma, both of which have malignant potential. Gonadectomy is recommended in XY gonadal dysgenesis to decrease risk of malignant transformation from gonadoblastoma. Although rare in pediatric patients, ovarian malignancies have been identified among AT cases. To our knowledge, we present the first case of AT due to borderline ovarian mucinous tumor of the ovary and contralateral gonadoblastoma in a patient with mixed gonadal dysgenesis (45,X/46,XY) and a Turner phenotype.

Meigs Syndrome Caused by Ovarian Granulosa Cell Tumor: A Case Report

Ovarian granulosa cell tumor (GCT) is extremely rare in children and adolescents, especially along with Meigs syndrome (MS). We describe the case of a 12-year-old girl who was referred to our center for massive peritoneal effusions with evidence of a pelvic mass on ultrasonography and computed tomography. The patient was treated with laparoscopic surgery (fertility-sparing surgery) and postoperative chemotherapy. Our case highlights the clinical importance of assessing MS in the diagnosis of pediatric female patients with peritoneal effusion and ovarian mass and the importance of fertility-sparing surgery.

Cervical Cancer Screening of Adolescents and Young Women: Further Evidence Shows a Lack of Clinical Value

To assess the prevalence of cytological abnormalities among young people from a large population in the city of São Paulo (Brazil). Retrospective, observational analysis of data from the institution's data processing center. A private laboratory in São Paulo (Brazil). Comparison of 3 different groups (ie, adolescent women [aged ≤19 years], young adult women [aged between 20 and 24 years], and adult women [aged 25 years and older]). Assessment of results from all cervical-vaginal smears collected for cytology between January 2010 and December 2015. Comparative analysis of cytological abnormalities in the 3 different groups. A total of 1,026,671 satisfactory cytology tests were performed. The proportion of cytological abnormalities was found to decrease with age (P < .001) and was similar in the groups comprised of adolescents and young adults, with 3.405/ 20.921 (16.3%) and 13,635/ 78,277 (17.4%), respectively, and 74,320/ 927,473 (8.0%) in the group of adult patients (P < .001). Among the positive cytologies in the group of adolescents, 3,331/ 3,405 (97.8%) represented low-grade lesions and 74/ 3,405 (2.2%) high-grade lesions, whereas among adults older than 25 years old, these figures were 69,092/ 74.320 (93%) and 5,228/ 74.320 (6.9%), respectively. No cases of cancer were found in the group of adolescents. Cytological screening of young people is not recommended because of the low prevalence of high-grade cytological abnormalities in this population, with cancer being a rare event. This inadvertent screening could lead to unnecessary complementary exams and overtreatment, which could compromise the reproductive future of these young women.

Squamous Carcinoma of the Cervix in a 15-Year-Old with Congenital HIV: A Case Report

Human papillomavirus (HPV) is the most prevalent sexually transmitted infection (STI) worldwide. Immunocompetent hosts have intact defense mechanisms to prevent HPV infection, but immunocompromised patients are at higher risk for complications, including HPV-related cancers. Most of these cancers originate from high-risk HPV strains in sexually active patients. Here we present a case of an immunocompromised adolescent who developed cervical cancer despite no prior sexual activity and only ever having had low-risk type HPV on biopsy. To our knowledge, this is the first case report of a cervical cancer arising from a low-risk HPV strain in an immunocompromised, non-sexually active adolescent. This case highlights the importance of preventive and screening mechanisms in immunocompromised populations, as they are have a higher probability of HPV-related complications, even in the absence of traditional risk factors.

Somatic DICER1 Mutations in a Pubertal Girl with Cervical Embryonal Rhabdomyosarcoma and Papillary Thyroid Adenoma

DICER1 syndrome is an inherited tumor predisposition syndrome. A germline mutation in DICER1 increases the risk for a spectrum of rare tumors. We describe a case of somatic DICER1 mutation in a pubertal girl, who is affected by different tumors of the DICER1 syndrome, including embryonal rhabdomyosarcoma (ERMS) of the cervix and thyroid adenoma. A 16-year-old girl with history of papillary thyroid adenoma presented with abnormal vaginal bleeding and an exophytic cervical mass on examination. Histopathologic examination confirmed cervical ERMS. By using Sanger sequencing of the tumor, we identified 1 DICER1 mutation (c.3937delG). This report suggests that a patient with these 2 unrelated tumors (ERMS and papillary thyroid adenoma) should be promptly tested for DICER1 gene mutations.

Successful Pregnancy after Intervention for Spindle-Cell Embryonal Rhabdomyosarcoma: A Case Report

Embryonal rhabdomyosarcoma, the most common soft tissue malignancy in childhood, is treated with surgery and chemotherapy. Because of the young age at the time of presentation, a discussion of future reproduction is appropriate and conservative management should be considered. We present a case of embryonal rhabdomyosarcoma that was successfully and conservatively managed with chemotherapy, allowing for future pregnancies. A 17-year-old nulliparous woman with embryonal rhabdomyosarcoma underwent 6 cycles of chemotherapy with adriamycin, dacarbazine, cyclophosphamide, and vincristine, resulting in radiographic resolution of the disease. She was able to conceive without medical intervention and to have successful vaginal deliveries. The standard of care for embryonal rhabdomyosarcoma is surgery and chemotherapy; however, conservative management should be considered when preservation of fertility is a goal.

A Rare Interlabial Mass in a 9-Month-Old Infant: A Case Report and Review of Literature

Interlabial masses in infants and children are quite rare. One of their rarest causes is urethral polyp or urethral caruncle. It is a benign fleshy outgrowth at the urethral meatus. Certain etiology is still unknown. A healthy 9-month-old female infant presented with a mass protruding from the vulva with no other complaints. Examination with the patient under general anesthesia revealed an interlabial mass appearing as a pedunculated pinkish polyp, originating from the posterior lip of the external urethral meatus. Surgical excision of the mass was done and histopathology confirmed it to be a urethral polyp. Urethral polyps are rare in the pediatric age group. Their occurrence in this age group might support a congenital etiology. Surgical resection of polyps allows histopathological examination and a high cure rate with no risk of recurrence.

Vulvar Hibernoma: An Unusual Lipomatous Tumor in an Adolescent

Lipomatous tumors of the vulva are exceedingly rare, particularly in adolescents. We describe the work-up and management of an adolescent girl who presented with a large, well-vascularized vulvar mass. A 14-year-old girl presented with a large vulvar mass of unclear etiology. Magnetic resonance imaging of this mass revealed an ill-defined, well-vascularized mass with fat signal characteristics suggestive of a lipomatous tumor that was concerning for malignancy. We performed complete resection of the mass, and histologic evaluation revealed a vulvar hibernoma. There have been no signs of recurrence 1 year later. Although rare, a hibernoma of the vulvar region may present in adolescence and may be concerning for malignancy on imaging. Complete resection of these tumors is recommended for definitive diagnosis and treatment.

A Rare Case of Vulvar Superficial Angiomyxoma in a Pediatric Patient

Superficial angiomyxoma (SAM) is a rare, benign cutaneous tumor. Originally described as a component of Carney complex, it is now recognized as a sporadic condition. A 7-year-old girl was referred for management of a 2.5-cm mass arising from the right labia majora. Key pathologic features included lobules of spindle-shaped cells in a myxoid matrix and prominent neutrophilic infiltrate. The cells were positive for CD34 and negative for desmin, progesterone receptor, and estrogen receptor staining. This case is that of the youngest described patient with vulvar SAM. Patients should be carefully examined for manifestations of Carney complex to avoid potentially life-threatening complications. It is critical to distinguish SAM from aggressive angiomyxoma. The patient was referred to genetics and will be followed for local recurrence.

Endometriosis in a Prepubertal Patient with 46,XY Difference in Sex Development: A Case Report

Endometriosis typically presents in postmenarchal patients with cyclic and acyclic pelvic pain. However, there are reports of endometriosis in premenarchal patients. We report a 10-year-old individual with 46,XY difference of sex development who was found to have endometriosis at the time of laparoscopic gonadectomy for gonadoblastoma. Although rare, endometriosis can occur in 46,XY individuals prior to puberty, highlighting the complex origin of the disease.

Sexual and Reproductive Health Care after Gonadotoxic Treatment in Females at a Tertiary Pediatric Hospital

Ovarian Leydig Cell Tumor Associated with Recurrent Torsion and Virilization in an Adolescent Patient

Ovarian tumors are rare in children; however, their incidence increases with age. Of these ovarian tumors, Leydig cell tumors are some of the rarest, accounting for less than 0.1% of all ovarian tumors across all ages. Leydig cell tumors predominantly occur in postmenopausal women and are characterized by nodular proliferation of Leydig cells in the ovarian hilum with intracytoplasmic Reinke crystals. These tumors secrete androgens, which can disrupt ovarian function, clinically presenting with abnormal uterine bleeding and virilization. Although they are generally benign, current recommendations are for treatment with a unilateral salpingo-oophorectomy. In adolescents, hyperandrogenism is most commonly caused by polycystic ovarian syndrome (PCOS); however, the differential for hyperandrogenism is broad. We present a case of a 15-year-old girl with a history of primary amenorrhea who presented with a Leydig cell tumor associated with recurrent ovarian torsion and virilization. This case reviews the challenges with diagnosis, management, and future implications of a rare androgen-secreting tumor in young patients.