Journal of Pediatric Surgery

Massive, benign, cystic ovarian tumors: A technical modification for minimally invasive resection

The majority of large, cystic ovarian tumors presenting in children are benign and amenable to ovarian sparing surgery (OSS). Laparoscopy is impractical in these cases and when attempted has been associated with a high rate of intraperitoneal fluid spill. We present a modified technique for controlled cyst decompression that allows delivery of the ovary through minilaparotomy and subsequent OSS. Criteria that must be met for the procedure to be undertaken are discussed.

Operative management of pediatric ovarian tumors and the challenge of fertility-preservation: Results from the UK CCLG Surgeons Cancer Group Nationwide Study

Ovarian tumors in the pediatric age group are rare. A significant number of children with ovarian mass lesions present "out of hours "as surgical emergencies, and surgical management does not always involve a surgical oncologist. This multicenter study reports how the mode of clinical presentation may influence (i) operation (conventional open vs minimally invasive surgery (MIS)) and (ii) examines if young females presenting as surgical emergency(s) are more likely to undergo total oophorectomy or ovarian sparing surgery. Retrospective multicenter study amongst UK pediatric surgical oncology centers. Females <16 years with diagnosis of ovarian tumor from 2006 to 2016 were included. Functional/neonatal ovarian cysts were excluded. Three hundred ten patients with ovarian tumors treated at 12 surgical oncology centers were identified. Mean age at surgery was 11 years [IQR 8-14]. Most common diagnosis were mature teratoma (57%, 177 cases), immature teratoma (10.9%, 34 cases) and cystadenoma (12%, 36 cases). Seventy percent (217) of cases were performed as open procedures. Thirty percent (94) of children underwent MIS. Tumors were significantly smaller in children who underwent MIS. Median tumor size in the laparoscopic group was 6 cm compared to 11 cm in the open group (p < 0.00001). Children who underwent MIS were significantly more likely to have ovary sparing surgery. This UK nationwide study demonstrates that ovary-sparing surgery and minimally invasive surgery are still infrequently deployed by pediatric surgeons in the UK. Patients with smaller tumors were more likely to undergo MIS, and more frequently underwent ovary-sparing surgery. In view of the implications on fertility and hormonal health caused by unilateral oophorectomy, it is time to review this current practise and agree consensus guidelines to reduce rates of unnecessary oophorectomy. This is a level II evidence study. It is a retrospective multicentre collaborative study, which summarizes data from a national cohort of children.

Ovarian tumors in children: how common are lesion recurrence and metachronous disease? A UK CCLG Surgeons Cancer Group nationwide study

Ovarian tumors in children are rare, mature teratoma being the most common histological entity. Robust guidelines to aid patient follow-up after resection are distinctly lacking. Although mature teratoma has a very good prognosis following complete resection, small studies have reported the occurrence of metachronous disease and recurrence to a variable degree (2.5-23% of patients). Nevertheless, there are surgeons who recommend no follow-up is required for these children after primary tumor resection. We investigated the incidence of (i) recurrence and (ii) metachronous disease in pediatric patients following ovarian tumor resection. Retrospective multicenter study amongst UK pediatric surgical oncology centers. Females <16 years with diagnosis of ovarian tumor from 2006 to 2016 were included. Functional/neonatal ovarian cysts were excluded. Three hundred ten patients with ovarian tumors treated at 12 surgical oncology centers were identified. Mean age at surgery was 11 years [IQR 8-14]. Most common diagnosis were mature teratoma (57%, 177 cases), immature teratoma (10.9%, 34 cases) and serous cystadenoma (7.7%, 24 cases). 8.1% (25 cases) of all females were identified with tumor recurrence/ metachronous disease. 5.1% (9 cases) of patients with mature teratoma had recurrent/ metachronous disease. Most of these patients were diagnosed at routine clinic follow-up. Our study clearly shows that ovarian tumor recurrence(s) and metachronous disease occur, even in "benign" ovarian tumors. We recommend female pediatric patients should have robust follow-up care plans after primary diagnosis and resection of ovarian tumor(s). This is a level II evidence study. It is a retrospective multicentre collaborative study which summarizes data from a national cohort of children.

Approaches to the management of pediatric ovarian masses in the 21st century: Systematic review and meta-analysis

Laparoscopy is increasingly being adopted for the treatment of ovarian pathologies in adults. However, its implementation for the management of pediatric ovarian masses varies and the evidence, to date, has not been comprehensively analyzed. This review aims to compare laparoscopic and open surgical management of pediatric ovarian masses. We searched PubMed, Cochrane Library and Google Scholar from the year 2000 till April 2017. Studies selected for this included those on epidemiological trends of pediatric ovarian lesions, assessing outcomes of laparoscopic management and comparison of laparoscopic and open surgical techniques for pediatric ovarian masses. A meta-analysis comparing outcomes of both modalities was performed using standard methodology. A total of 44 studies met the inclusion criteria of which 15 were on histological types of ovarian lesions, 24 assessed laparoscopic management only and five compared laparoscopy with open surgery for pediatric ovarian masses. Nonneoplastic lesions were the most common ranging from 36.5% to 73.7%, with cystic lesions being the most prevalent. Neoplastic lesions ranged between 26.3% and 63.5%, with germ cell tumors being the most common, while malignancy ranged between 3.5% and 10.8%. Laparoscopic management was generally advocated for managing benign lesions with a cautious approach for suspicion of malignant lesions. In comparison to open surgery, laparoscopic surgery had shorter operating time (MD = -33.24 min, 95% CI = -34.29 to -32.19, p < 0.0001), less intraop bleeding (MD = - 61.46 ml, 95% CI = -62.69 to -60.24, p < 0.0001), and reduced length of hospital stay (MD = -2.78 days, 95% CI= -2.82 to -2.74, p<0.0001). Complication rates were equivocal between the two approaches. Spillage rates could not be assessed. Limited evidence suggests that laparoscopic approach to presumptively benign ovarian masses have better outcomes when compared to open surgery with regards to operating time, blood loss and hospital stay. However, complication rates were similar between the two approaches. Studies with rigorous scientific methods are needed for a definitive recommendation, especially in resource limiting settings. However malignant lesions should still be managed with an open surgical approach to avoid upstaging of disease status. II.

Understanding the Value of Tumor Markers in Pediatric Ovarian Neoplasms

The purpose of this study was to determine the diagnostic accuracy of tumor markers for malignancy in girls with ovarian neoplasms. A retrospective review of girls 2-21 years who presented for surgical management of an ovarian neoplasm across 10 children's hospitals between 2010 and 2016 was performed. Patients who had at least one concerning feature on imaging and had tumor marker testing were included in the study. Sensitivity, specificity, and negative and positive predictive values (PPV) of tumor markers were calculated. Our cohort included 401 patients; 22.4% had a malignancy. Testing for tumor markers was inconsistent. AFP had high specificity (98%) and low sensitivity (42%) with a PPV of 86%. The sensitivity, specificity, and PPV of beta-hCG was 44%, 76%, and 32%, respectively. LDH had high sensitivity (95%) and Inhibin A and Inhibin B had high specificity (97% and 92%, respectively). Tumor marker testing is helpful in preoperative risk stratification of ovarian neoplasms for malignancy. Given the variety of potential tumor types, no single marker provides enough reliability, and therefore a panel of tumor marker testing is recommended if there is concern for malignancy. Prospective studies may help further elucidate the predictive value of tumor markers in a pediatric ovarian neoplasm population. Retrospective Cohort Review. Level III.

Pediatric ovarian immature teratoma: Histological grading and clinical characteristics

Ovarian immature teratomas (ITs) are relatively rare among all pediatric ovarian tumors. The histological grading for ovarian ITs, which ranges from 1 to 3, is based on the proportion of immature neuroepithelial component. Higher-grade ITs in adults are treated as malignant neoplasms and require adjuvant chemotherapy. However, there is no consensus on the therapeutic management of pediatric ovarian ITs. The aim of our study was to analyze the histological grades and clinical characteristics of ovarian ITs in pediatric patients. This retrospective chart review consisted of seven patients, including one, three, and three patients with histological grade 1, 2, and 3 pediatric ovarian ITs, respectively, who were treated at our institute between 2000 and 2016. Collected data comprised age, alpha-fetoprotein (AFP) level, clinical stage, tumor size, treatment, and prognosis. The median age and AFP levels of patients with grade 1, 2, and 3 ovarian ITs were 8, 7, and 10 years and 37, 112, and 221 ng/ml, respectively. All cases were Children Oncology Group (COG) stage I and International Federation of Gynecology and Obstetrics (FIGO) stage IA. All patients had unilateral tumors in the right ovary. The median tumor sizes of the grade 1, 2, and 3 IT patients were 104, 160, and 100 cm Clinical characteristics of patients with grade 3 ovarian ITs were relatively older and had higher AFP levels than those with lower-grade ITs. According to our patient's clinical course and prognosis, COG stage I pediatric ITs should be treated by surgery alone and that postoperative chemotherapy is unnecessary even for those with grade 3 ITs as well as patients with rather low AFP levels. IV.

Controlled aspiration of large paediatric ovarian cystic tumours

Cystic ovarian masses in children may be physiological or neoplastic. It is mandatory that suspected neoplastic lesions are resected without tumour spillage. However, a large midline incision is cosmetically unappealing incision to young women. Here we describe our experience of using controlled drainage without spillage that allows a cosmetic pfannenstiel approach without compromising oncological principles. All girls treated with large ovarian cystic masses since 2008 in our centre were identified and data was collected prospectively. A small pfannenstiel incision was performed followed by peritoneal washings; tissue glue was used to stick an Opsite™ dressing to the cyst surface and fluid drained so there was no leakage back into the patient. Once aspirated the cyst was delivered and an ovarian preserving cystectomy was performed where possible. Twenty-three girls (median age 14.5 years (8.1 to 16.5 years) were included. Pre-operative MRI scan showed a complex lesions with median volume of 1169 ml (range 252-7077 ml). At surgery 22/23 cysts were intact and removed without spillage. mature teratoma (11), serous cyst (3), mucinous cyst adenocarcinoma (2), mucinous cystadenoma (5), Sertoli-Leydig tumour, sclerosing stromal tumour. One girl with pre-operative rupture of a mucinous adenocarcinoma subsequently died. Ovarian sparing cystectomy was performed in 17/23 girls. All other patients are well without evidence of recurrence. This is the largest series in children and adolescents using controlled drainage of cystic ovarian tumours. Though there were a range of diagnoses we have shown that these can be removed safely with a cosmetic pfannenstiel approach while following oncological principles.

Ovarian sparing surgery can be achieved for many pediatric patients with giant adnexal cystic masses

Ovarian-sparing surgery (OSS) is the surgical treatment of choice for benign ovarian tumors in children, but OSS can be technically challenging in the setting of giant ovarian cystic masses as ovarian tissue can become thinned or stretched. We aim to clarify if OSS is a feasible approach to giant ovarian cystic masses in children and if morphologically normal appearing ovarian tissue can be detected on post-operative imaging following OSS. We performed a retrospective review of all adnexal surgical procedures at a single children's hospital from 2015 to 2023. Children undergoing surgery for an adnexal cystic mass ≥15 cm in size were included. We report surgical approach, identification of ovarian tissue on post-operative ultrasound, and recurrence rate. Univariate analysis was performed for all data points. We identified 55 patients who underwent surgery for adnexal cystic masses ≥15 cm. The median age of patients was 15.0 years (Range: 10.4-20.5 years). Of the 55 patients, 40 (72.7 %) were pre-operative candidates for OSS. Of these 40 patients, 25 (62.5 %) underwent OSS. The remaining 15 (37.5 %) had either significantly thinned and unidentified ovarian tissue intraoperatively (N = 11), non-viable ovarian torsion (N = 1), adhesive disease preventing OSS (N = 1), intra-operative concern for malignancy (N = 1), or elected for oophorectomy (N = 1). All 22 patients who underwent post-operative doppler ultrasound following OSS had morphologically normal appearing and perfused ovarian tissue on imaging. OSS resulted in 1 local recurrence of serous cystadenoma. Children with giant ovarian cystic masses and a benign pre-operative risk stratification are a population of patients with opportunity for meaningful ovarian salvage. IV - Retrospective Cohort Study.

Racial Disparities in Pediatric Ovarian Mass Management

Race, ethnicity, gender and social determinants of health have been found to impact access to pediatric surgical care and outcomes. However, there is limited research examining the effect of patient race on surgical intervention in pediatric females presenting with ovarian masses. A retrospective single-institution cross-sectional review between 2009 and 2021 was performed which included individuals with sex assigned as female at birth ≤18 years of age who underwent surgery for an ovarian mass. Socio-demographics, self-reported race, preoperative imaging and laboratory assessment, type of surgery, and final pathology were collected. Fisher's exact test was used to determine if clinical findings among three race groups and two social determinants of health metrics were different. The study included 409 distinct adnexal mass cases. 196 (48 %) of patients identified as White race, 159 (41 %) identified as Black race, and the remaining 44 (11 %) were labelled as Other race. Overall, 383 (93.6 %) cases had benign pathology and there was no difference in rate of malignancy on final pathology by race group (p = 0.862). Black patients were significantly more likely to undergo oophorectomy compared to White patients (p = 0.009). There were no differences in pre-operative evaluation, surgical management, or final pathology based on social determinants of health indices. Black females were significantly more likely to undergo oophorectomy compared to White females despite no differences rates of malignancy. The implementation of a preoperative risk stratification algorithm may mitigate racial biases in this population, however, further investigation is needed to address this disparity. Level 2.

The Shared Ovary: A Multidisciplinary Discussion With Pediatric and Adolescent Gynecology

Pediatric and adolescent ovarian lesions are common and are frequently managed by both pediatric surgeons and pediatric and adolescent gynecologists. During the 2023 American Academy of Pediatric Section on Surgery meeting, an educational symposium was delivered focusing on various aspects of management of pediatric and adolescent benign and malignant masses, borderline lesions, and fertility options for children and adolescents undergoing cancer therapies. This article highlights the discussion during this symposium.

Impact of Surgical Timing (Primary, Delayed, or Second Look) on Surgical Morbidity and Outcomes in Malignant Germ Cell Tumor of the Ovary in Children

Malignant ovarian germ cell tumors (MOGCT) are rare in children. Surgery with or without chemotherapy is the primary treatment approach. This study aimed to analyze the impact of primary and delayed surgery on surgical morbidity and outcomes. Second-look surgery after inadequate surgical staging and the various components of surgical staging were also evaluated. Children below 15 years with MOGCT treated between 2006 and 2022 were analyzed. A comparison of patients undergoing primary, delayed, and second-look surgery was performed. 118 patients with a median age of 12 (0.11-15) years were eligible. Forty patients underwent primary, 51 delayed, and 27 second-look surgeries. Overall complications, including tumor rupture, blood loss, and adjacent organ removal, were significantly higher in the primary compared to the delayed surgery group (p = 0.0001). Second-look surgery conceded more blood loss (p = 0.0001), extended duration (p = 0.03), and complications (p = 0.004) than delayed surgery. The compliance with surgical guidelines was 100% for most components, with a positive yield rate of 10-80%. At a median follow-up of 5.2 years, the 5-year event-free survival (EFS) and overall survival (OS) for the entire cohort are 86% and 89%, respectively. The OS and EFS did not differ by the timing of surgery, although the second-look surgery demonstrated relatively inferior outcomes consequential to initial suboptimal surgery. MOGCT shows favorable outcomes. Delayed surgery after chemotherapy in appropriately selected patients minimizes the morbidity of surgery with similar outcomes compared to primary surgery. An optimal initial surgery is essential since second-look surgery produces significant morbidity. Prognosis Study, Level II evidence.

Laparoscopic Ovarian-Sparing Surgery for the Management of Benign Ovarian Lesions in Pediatric Patients: A Retrospective Analysis

Laparoscopic ovarian-sparing surgery (OSS) is safe and effective management approach for benign ovarian lesions in pediatric patients. This study evaluates the outcomes of females younger than 18 years who underwent the OSS procedure between December 2013 and November 2022 at a single institution. We conducted a retrospective analysis of records from 82 females who underwent OSS for ovarian lesions. OSS was performed based on diagnostic imaging that suggested the benign nature of the lesion. Of the 82 patients studied, 78 had unilateral lesions and 4 had bilateral synchronous lesions. The mean age was 14 years. The majority (62 cases) of the surgeries were laparoscopic, with 20 requiring conversion to open surgery due to factors such as indistinguishable edges and large size of the lesion. We identified 8 cases of ovarian torsion. The surgical specimens revealed that 46 were ovarian teratomas, 2 were granulosa cell tumors, 15 were cystadenomas, and 23 were functional cysts. There were no intraoperative complications. Two recurrences were observed in patients who were initially treated for bilateral ovarian teratomas. One patient developed a pelvic abscess. Additionally, three patients had metachronous ovarian tumors during the follow-up period. In patients followed with ultrasound imaging, the viable ovary was visualized in 83.6% of the cases (61 out of 73). Our findings demonstrate the effectiveness of laparoscopic OSS in preserving ovarian function and providing clinical benefits in patients with benign ovarian lesions. We recommend regular follow-up with ultrasound to exclude metachronous lesions or recurrence. III.

Contemporary Trends in Laparoscopy and Ovarian Sparing Surgery for Ovarian Torsion in the Pediatric Population

Although total oophorectomy (TO) was historically performed in cases of nonviable-appearing ovaries, considerable evidence has demonstrated equivalent outcomes after ovarian sparing surgery (OSS) as well as long-term fertility preservation benefits. This study sought to compare outcomes of OSS and TO for patients with ovarian torsion. Females 50 covariates (demographics, medical comorbidities, ovarian diagnoses, etc.) was constructed between those receiving TO and OSS. There were 3,161 females (median 15 [12-18] years) with ovarian torsion, and concomitant pathologies included cysts (42%), benign masses (25%), and malignant masses (<1%). Open approaches were more common (52% vs. 48% laparoscopic), and ovarian resection (OSS or TO) was performed in 87% (39% OSS and 48% TO). OSS was more commonly performed with laparoscopic detorsions (60% vs. 40% TO), while TO was more frequent in open operations (59% vs. 41% TO; both p < 0.001). No differences in overall readmissions (7% OSS vs. 8% TO) or readmissions for recurrent torsion (<1% overall) and ovarian masses (<1%) were observed (both groups <1%; p = 0.612). After PSMA, laparoscopy was still utilized less frequently with TO (39% vs. 53%; p < 0.001) despite similar rates of malignant masses. Overall, these data offer additional support for the current practice guidelines that give preference to OSS as the primary method of treatment for pediatric ovarian torsion in the majority of cases. III. Retrospective Comparative Study.

Letter to Editor in Response to: Controlled Aspiration of Large Paediatric Ovarian Cystic Tumors

Laparoscopy versus laparotomy for pediatric ovarian dermoid

Approaches to the Diagnosis and Management of Paediatric Ovarian Tumours and Oncological Outcomes in a Single-centre Study. Evidence in Support Of IPSO Reccomendations

Approaches to paediatric ovarian tumours vary. Cross-sectional imaging is reliable but not always performed. Laparoscopic tumourectomy, although popular, is not recommended by International Paediatric Surgical Oncology Society (IPSO); tumour spillage remains the main concern. We aimed to investigate the reliability of preoperative evaluation and identify perioperative factors associated with adverse outcomes. Single-centre retrospective study (2015-2022) of clinical presentation, preoperative investigation, operative approaches and oncological outcomes in all females <18 years treated for ovarian tumours. Logistic regression was used to identify predictors of malignancy and of second disease events. Data are presented as median (IQR) or rates. Sixty girls aged 12.5 (10-15) years with follow-up of 51 (30.5-76.3) months were included. Incidence of malignant and low malignant potential tumours was 19/60 (32%). Tumour size and consistency proved predictive of malignancy. Clinical assessement, tumour markers and cross-sectional imaging combined showed 75% sensitivity, 100% specificity and 7% misclassified malignancy rate. Second disease events (11/60, 18%) were associated with misclassified malignancy (2/11 vs. 1/49, p = 0.04) and positive margins (4/11 vs. 5/49, p = 0.02). Ovarian-sparing surgery did not influence second events (5/11 vs. 28/49, p = 0.48). There was no significant association between second events and laparoscopic tumourectomy (3/11 vs. 20/49, p = 0.40); spillage rate was however more frequent with laparoscopy (5/11 vs. 2/32, p = 0.008). Misclassified malignancy, noted in 7% of patients undergoing optimal work-up, adversely impacted outcomes, highlighting the importance of oncological principles in both oophorectomy and ovarian-sparing surgery. Malignancy incidence and misclassification can be underestimated when tumours with malignant behaviour potential, such as immature teratomas and borderline tumours, are grouped as benign. There was no direct association between operative approaches and second events. Laparoscopy, however, failed to maintain oncological principles more frequently than open surgery and hence risks upstaging of paediatric ovarian pathology. III.

Medium-term Ovarian Volume and Anti-Müllerian Hormone After Ovarian-sparing Surgery for Benign Neoplasms in Pediatric Patients

Benign ovarian neoplasms are common in the pediatric population. In young adult women, oophorectomy has been shown to negatively impact long-term ovarian endocrine function. Recently, ovarian-sparing surgery (OSS) has been proposed as it offers similar results to oophorectomy in terms of recurrence rates. However, the impact of OSS on functional preservation in girls and adolescents remains unclear due to limited follow-up data. The aim of this study was to evaluate ovarian function and its morphological correlation in the medium term in girls undergoing OSS for benign neoplasms. A retrospective cohort study was conducted including girls under 15 years of age who underwent OSS for benign neoplasms at our hospital between January 2014 and July 2022. Eligible patients were cited to an on-site evaluation at least 12 months after surgery. Data collected included age at surgery, tumor volume, history of adnexal torsion and recurrence. Postoperative ovarian volume was assessed by transabdominal pelvic ultrasound, and serum anti-Müllerian hormone (AMH) levels were measured and compared to expected values for age. Thirty-seven patients presented with 39 ovarian neoplasms; 31 underwent OSS. Mean age at surgery was 11.7 years, and mean period from surgery to face-to-face evaluation was 46.6 months. Excluding cases with ovarian torsion, normal ovarian volume (>-2 standard deviations (SD) from the expected value for age) was observed in 22/27 ovaries (81.5 %). In patients with normal volume ovaries after unilateral disease, 17/19 (89.5 %) AMH values were > -1SD from the expected value. A positive and statistically significant correlation was found between postoperative ovarian volume and AMH levels (Pearson correlation 0.52, p < 0.01). One patient who underwent oophorectomy and one who underwent OSS reported achieving pregnancy without medical intervention. No ipsilateral recurrences were observed during the follow-up period. OSS is a viable alternative for treating benign neoplasms in girls, showing morphological and functional results comparable to healthy women from one-year post-intervention onwards. Long-term follow-up and prospective studies are needed to determine the effectiveness of OSS in maximizing fertility and avoiding premature menopause, which occurs after oophorectomy at an early age, while maintaining similar oncological outcomes. IV.

Female Fertility Cryopreservation Outcomes in Childhood Cancer: A Systematic Review