Journal

Pediatric Surgery International

Papers (18)

Vaginal and cervical tumors in children and adolescents: a SEER population-based study

Vaginal and cervical tumors in children and adolescents are rare and poorly understood. This study aims to provide a comprehensive analysis of incidence, treatment modalities, and survival outcomes of vaginal and cervical tumors in this age group using the SEER database. Data were retrieved from the SEER database (2000-2019) for patients aged 0-18 years diagnosed with primary vaginal and cervical tumors. Demographics, tumor characteristics, treatment modalities, and survival outcomes were analyzed. Kaplan-Meier survival analysis was used to estimate overall survival (OS). To examine the impact of each factor on overall survival, a multivariate Cox proportional hazards model was created. Based on the results of the Cox regression model, a nomogram was constructed. Between 2000 and 2019, 164 pediatric and adolescent patients with vaginal and cervical tumors were analyzed, with a median age of 14 years (range 0-18 years). Embryonal rhabdomyosarcoma (40.9%) being the most common subtype. The overall survival rates were 97.5% at 1 year, 91.1% at 3-year, and 88.9% at 5-year. Younger patients (0-14 years) had significantly better prognoses than older ones (15-18 years). Additionally, survival outcomes differed by histological subtype, with YSTs showing the highest survival rate (100%) and clear cell adenocarcinoma exhibiting the poorest prognosis. Distant metastases were associated with worse outcomes. Age and SEER stage were identified as independent predictors of survival, with a nomogram constructed to further predict outcomes. Vaginal and cervical tumors in children and adolescents have distinct demographic and clinical patterns. Younger age, localized disease, and specific histological subtypes, such as YSTs, are associated with favorable outcomes. Early diagnosis and individualized multimodal treatment are crucial for improving outcomes.

The role of preoperative imaging and tumor markers in predicting malignant ovarian masses in children

To investigate the clinicopathololgic characteristics and the predicting value of preoperative imaging and tumor markers in children with ovarian masses. Patients admitted in Shanghai children's hospital with ovary neoplasms between 2010.01 and 2015.12 were retrospectively analyzed. The medical records including age at operation, presentation of symptoms and signs, tumor marker, imaging, pathology, tumor diameter and surgical choice were reviewed. All data were analyzed using SPSS 17.0 RESULTS: A total of 139 patients were included, among which 116 were benign neoplasms and 23 malignant tumors. There was significance difference relation with the tumor diameter, character, torsion and tumor markers, but not the age, position, calculi, and symptoms. The risk factors include tumor diameters ≥ 10 cm, the odds ratio (OR) was 11, 95% confidence interval (CI) was 3-36, solid/complex tumor (OR 6, 95% CI 2, 14) and positive in tumor markers (OR 84, 95% CI 20, 345). Among the patients with benign neoplasms, 77 of them had laparoscopic ovarian cystectomy while 23 patients with malignant tumors had salpingo-oophorectomy and omentum resection. Preoperative imaging and tumor markers could help identifying the malignant ovarian masses in children. If tumor diameter ≥ 10 cm, solid/complex in imaging and tumor markers abnormal, a radical resection is mandatory; otherwise, an ovarian-sparing surgery is recommended.

The need for screening recommendations after intestinal vaginal replacement: a systematic review of the literature

To investigate the occurrence, presentation, management, and treatment of patients found to have a primary colorectal malignancy arising in the intestinal neovagina. A systematic review of primary research studies describing intestinal malignancy arising in an intestinal neovagina was conducted. Reports of extraintestinal malignancy and secondary research study designs were excluded. Sixteen case reports were identified over 35 years. Patients commonly had sigmoid (n = 11, 69%) neovaginas. Indications for vaginal reconstruction included Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome (n = 5, 31%), gynecologic carcinoma (n = 3, 19%), vaginal agenesis (n = 5, 31%) and cloaca (n = 3, 19%). Symptom onset ranged from 7 to 53 years after reconstruction. Age at diagnosis ranged from 17 to 53 years. Bleeding was the most common presenting symptom (n = 12, 73%). Fifty percent of patients presented with local disease (n = 8), 31% with locoregional (n = 5) and 6% with metastasis (n = 1). Treatment varied with most patients undergoing surgical resection. Survival was reported for 10 patients, half of which experienced disease recurrence. Surveillance plans were rarely described. Adenocarcinoma in intestinal neovaginas is a rare but life-threatening disease that lacks routine screening, management and surveillance guidelines. Our findings underscore the variability in management of these patients, and the morbidity of the disease. Work to develop formalized screening guidelines is ongoing.

Vaginal tumours in childhood: a descriptive analysis from a large paediatric medical centre

Malignant tumours of the vagina are very rare in children. The purpose of this study was to retrospectively analyse the clinical presentation, treatment, and outcome of vaginal tumours in children treated in a single institution. This study retrospectively analysed the clinical data of children diagnosed with vaginal malignant tumours who were admitted to the Beijing Children's Hospital of Capital Medical University from January 2007 to December 2020 and followed these patients to observe their prognoses and outcomes. During 13 years, a total of 33 children were included in this study, including 13 children with rhabdomyosarcoma and 20 children with endodermal sinus tumours. The average age at diagnosis was 20.4 months. The main clinical manifestations were vaginal bleeding and protruding masses. Of the 13 children with vaginal rhabdomyosarcoma, 12 were treated with multidrug chemotherapy combined with conservative tumour resection, and their tumours completely resolved; only one child underwent vaginectomy and hysterectomy. Twenty children with vaginal endodermal sinus received PEB chemotherapy. Among these patients, the tumour disappeared after chemotherapy in 12 children, and the remaining nodular tumour foci in 8 children were confirmed to be necrotic tissue by pathology. Our research confirms that chemotherapy combined with conservative surgical treatment is effective for treating children with vaginal malignancies.

Surgical management and outcomes of paediatric ovarian tumours—a 25-year UK single centre experience

Ovarian neoplasms in children are rare heterogeneous lesions with variable biological behaviour and outcome. We herein report a 25-year experience from a UK specialist centre managing paediatric ovarian tumours. All female patients requiring operation for ovarian tumours were identified during the study period 1990-2018. Patient demographics, clinical presentation, and management are highlighted. Eighty-eight females with 101 ovarian tumours including cases with synchronous and metachronous neoplasms were documented. Mean age at diagnosis was 11.8 years. Tumours were equally distributed in left and right ovary(s). Bilateral disease occurred in 11 (10.8%) patients. Eighty-six (85%) neoplasms were benign and 15 (15%) malignant requiring in addition to surgery chemotherapy and/or radiation. There were no significant difference(s) observed in maximum tumour diameter in benign vs malignant lesions. Only half of malignant tumours had positive tumour markers. Fifteen patients (15%) had ovarian lesions that were metastatic at primary presentation including a rare case of disseminated peritoneal gliomatosis. Surgical management included salpingo-oopherectomy n = 21 (21%), ovary excision n = 33 (33%), 'ovary sparing' tumourectomy n = 34 (34%), and cyto-reductive extirpation in 2 cases (2%). Lymphadenectomy was performed in 9 patients (9%) together with peritoneal fluid cytology in 18 cases (18%) who had abdominal ascites. Omentectomy was required in 14 patients (14%), multiple peritoneal biopsy(s)-in 11 cases (11%) and biopsy of other suspicious involved organs notably bowel, gut mesentery and contralateral ovary in 7 (7%) further patients. Recurrent and metachronous lesions occurred in 10 (10%) cases in those with teratoma (n = 9) and adenocarcinoma(s) (n = 1). Overall patient survival was 97% with only 3 deaths-mucinous cystadenocarcinoma (n = 1), desmoplastic small round cell tumour of ovarian origin (n = 1), and a small cell carcinoma of ovary with hypercalcaemia (n = 1). Findings from this study show an excellent survival (97%) for paediatric ovarian neoplasms. Rare malignant neoplasms though do carry a poor prognosis. In the modern era of care, all efforts should be made to preserve future hormonal health and fertility with 'ovarian sparing' operation(s) where appropriate.

Effect of emergent nephrostomy on long-term total and split renal function in patients with upper urinary tract obstruction due to pelvic malignant tumors

Abstract Purpose This study aimed to investigate the impact of nephrostomies on the outcome of total renal function (TRF) and split renal function (SRF) in patients with malignant pelvic tumors associated with upper urinary tract obstruction (UUTO). Methods Patients with pelvic tumors suffering severe unilateral hydronephrosis treated at our hospital from 2000 to 2022 were included. Data for nephrostomy placement, short- and long-term renal function, and radiological and nuclear imaging studies were collected. The TRF and SRF of patients who underwent nephrostomy were compared to those who did not. Results Seven patients were included (rhabdomyosarcoma: 5, ovarian germ cell tumor: 1, malignant rhabdoid tumor: 1). Nephrostomies were placed in four, which were successfully managed without severe infections. Estimated glomerular filtration rate (eGFR) was significantly improved at the end of treatment in patients with nephrostomy. In contrast, eGFR in patients who did not undergo nephrostomy was not improved. Nuclear imaging studies (renograms or renal scintigrams) revealed impaired SRF of the affected kidney compared to the contralateral kidney, even in patients whose eGFR was within normal levels. Notably, SRF showed a trend to improve over time in one patient treated with nephrostomy. Conclusion Nephrostomy for UUTO caused by pelvic tumors may improve renal outcome.

Rate of oophorectomy in pediatric ovarian torsion: risk factors and change over time

Abstract Purpose The management of ovarian torsion in pediatric patients has evolved over time. Ovarian salvage is currently recommended given concerns for fertility preservation and the low likelihood of malignancy. Studies have shown that the incidence of oophorectomy is higher amongst pediatric surgeons in comparison to gynecologists. Using a national database, this study examined how the surgical management of ovarian torsion has evolved. Methods Children with a discharge diagnosis of ovarian torsion (ICD-9 code 620.5, ICD-10 code N835X) and procedure codes for oophorectomy (CCS code 119) were identified within the KID database from 2003, 2006, 2009, 2012, 2016, and 2019. Diagnosis of ovarian pathology was based upon ICD-9 and ICD-10 codes at the time of discharge. Results A total of 7008 patients, ages 1–20, had a discharge diagnosis of ovarian torsion. Of those patients, 2,597 (37.1%) were diagnosed with an ovarian cyst, 1560 (22.2%) were diagnosed with a benign ovarian neoplasm, and 30 (0.4%) were diagnosed with a malignant neoplasm. There was a decreased risk of oophorectomy in urban-teaching versus rural hospitals (OR: 0.64, p < 0.001). The rate of oophorectomy has decreased overtime. However, patients with benign or malignant neoplasms were more likely to undergo oophorectomy than those without a diagnosis (OR: 2.03, p < 0.001; 4.82, p < 0.001). Conclusion The rate of oophorectomy amongst children with ovarian torsion has decreased over time. Yet, despite improvements, oophorectomy is common amongst patients with benign ovarian neoplasms and those treated at rural hospitals. Continued education is needed to optimize patient care in all clinical scenarios. Level of evidence IV.

Direct balloon trocar technique for closed decompression of large predominantly cystic ovarian tumors in adolescents

Abstract Purpose Large ovarian tumors with a predominantly cystic pattern are uncommon in adolescents. This study evaluates the effectiveness of a decompression technique utilizing a balloon trocar to facilitate ovarian-sparing surgery, while preserving oncologic principles, in adolescent patients presenting with such lesions Methods A retrospective review of four consecutive cases managed by a single surgeon over a 7-year period ending December 2024. We describe the surgical technique of this innovative approach, and provide patient-specific clinical data including histopathologic findings and follow-up outcomes. Results The median patient age was 13 years (range, 10–17), and the median maximum tumor diameter was 17 cm (range, 10–28). The balloon trocar technique enabled a tight seal and closed suction drainage of the tumor cyst fluid, facilitating safe decompression and exteriorization of the mass. All surgeries were completed with negligible morbidity. Histological examination revealed three mature cystic teratomas and one serous cystadenoma. At clinical and ultrasonographic follow-up ranging 8 months to 7 years (median, 6,5 years), all patients remained free of recurrence, maintained regular menstrual cycles with normal-sized preserved ovaries, and expressed high satisfaction with their cosmetic outcomes. Conclusion Large predominantly cystic ovarian tumors are generally associated with a favorable prognosis. Preemptive in situ decompression using a balloon trocar represents a simple, effective, and rapid technique to minimize the risk of tumor spillage. This approach facilitates safe ovary-sparing surgery for these large tumors, while also providing excellent cosmetic outcomes.

“Floating ball sign” in the diagnostic imaging of mature ovarian teratomas in children

AbstractThe “floating ball sign” (FBS) is a rarely described visual phenomenon found in mature ovarian teratoma imaging. It is characterized by the presence of movable, spherical areas within the cystic component of a tumor. Such visualization is possible both in cross-sectional imaging and ultrasonography. To evaluate the incidence of FBS in the pediatric population with regard to patients’ age and tumor size. This is a retrospective study of pediatric patients operated on in a tertiary pediatric surgical center between January 2009 and December 2022 due to mature ovarian teratoma; the medical records were reviewed for the age at diagnosis, recurrences, tumor size, and their characteristics in preoperative imaging. Eighty-three patients (mean age 14, range 0–17) out of 91 met the inclusion criteria for the analysis. Eighty-seven operations on 90 ovaries were performed. Preoperatively 38 patients underwent CT, 13 MRI, and 39 received only the ultrasound examination. The FBS was identified in preoperative imaging diagnostics in 3 (3.3%) girls (14, 16 and 17 years of age). The average largest tumor dimension and volume were 142 mm and 1268 cc in the FBS group, and 73 mm and 252 cc in the remaining group, respectively. FBS tumors usually reach large sizes. Although the sign is rare in children, there are no scientific reports of its occurrence in the first life decade. Color flow mapping and cross-sectional imaging play a pertinent role in distinguishing this uncommon pattern from a malignant mass and enable the selection of an appropriate surgical approach.

Surgical resection and outcome of malignant ovarian germ cell tumors in children—a national multicentric study compared to international results

To evaluate the outcome of pediatric malignant ovarian germ cell tumors treated by three tertiary Egyptian institutions, and to compare our national experience to internationally published data. This is a retrospective analysis of all patients presenting between September 2014 and September 2019. Management protocol was Children's Oncology Group (COG) in all participating centers. Overall survival (OS), event-free survival (EFS) and univariate prognostic factors were estimated by Kaplan-Meier and log-rank test. Additionally, a review of various practices that reported survival outcome was conducted. Thirty-seven patients were included with a median age of 10.5 years (1-18 years). Thirty-five patients had unilateral salpingo-oophorectomy. Mixed germ cell and yolk sac tumors represented 75.7% of patients. There were 7 (19%), 14 (37.8%), 12 (32.4%) and 4 (10.8%) stage I, II, III and IV, respectively. Seven patients were low risk (LR), 26 intermediate risk (IR) and 4 high risk (HR). Platinum-based chemotherapy was administered as per risk stratification. Follow-up to March 2020 revealed that five patients had relapsed. There were no statistical significances of pathological types and patients' age regarding OS (p value 0.392 and 0.281, respectively) and EFS (p value 0.420 and 0.437, respectively). Three-year OS was 84%: 100% for stages I and II, and 62% for stages III and IV (p = 0.003); 100% for LR, 89% for IR, and 24% for HR (p < 0.001). Three-year EFS was 87%: 96% for stages I and II, and 71% for stages III and IV (p = 0.025); 100% for LR, 92% for IR, and 26% for HR (p < 0.001). Surgical resection combined with chemotherapy achieves excellent outcome for such tumors in both, present study and previous reports. On the basis of our results, COG staging and risk stratification were significantly correlated with prognosis, whereas tumor pathology and age had no significant impact. Prognostic factors are controversial among studies, and further research is still required.

Long-term surgical outcomes in pediatric ovarian neoplasms: 20-year single-center experience

The management of pediatric ovarian neoplasms (ON) is based on finding a balance between adequate surgical treatment and future reproductive capacity. We aimed to evaluate long-term results of patients who underwent surgery for ON. A retrospective cohort study design was used. Medical records of patients with ON were reviewed. They were invited to participate in a telephone-based survey assessing complaints, menstrual status, and post-surgical recurrence. Eighty-five patients were operated for ON between 1995 and 2015. Median age at surgery was 14.7 years. 62.4% of patients had ovary-sparing surgery (OSS). Median tumor size in oophorectomy group was significantly larger than OSS group (p = 0.029). Median length of follow-up was 5.1 years. Recurrent/metachronous disease was not significantly different between OSS and oophorectomy groups (p = 1.000). In OSS group, irregular menses (p = 0.004) and painful menses (p = 0.002) were significantly higher than oophorectomy group. The main goal of treatment in pediatric ON is to find the right balance between adequate and appropriate tumor resection and maximal effort for fertility preservation. Our results showed no difference between oophorectomy and OSS in the terms of recurrence. Although irregular and painful menses were found to be significantly higher in the OSS group, longer follow-up and prospective studies are needed to clarify this issue.

Save the ovary: evaluating the diagnostic accuracy of the pediatric risk of malignancy index (PRMI) to prevent unnecessary oophorectomy in girls

This study aimed to validate the diagnostic accuracy of the Pediatric Risk of Malignancy Index (PRMI) for distinguishing benign from malignant ovarian lesions in the pediatric and adolescent population, and to model its potential role in avoiding unnecessary oophorectomies. We conducted a retrospective study of females aged ≤18 years who underwent oophorectomy with histopathology between 2006 and 2016 at a UK tertiary hospital. Only patients with preoperative imaging were included. PRMI scores were retrospectively calculated, with a cut-off ≥7 indicating high malignancy risk. Diagnostic performance was assessed against final histology using standard accuracy measures. Of 265 ovarian specimens, 44 met inclusion criteria; 9 (20.5%) were malignant and 35 (79.5%) benign. PRMI showed a sensitivity of 77.8% (95% CI 45.2-94.4%), specificity 74.3% (95% CI 56.4-86.4%), positive predictive value 43.8%, and negative predictive value 92.9%. Positive and negative likelihood ratios were 3.03 (95% CI 1.45-6.35) and 0.30 (95% CI 0.09-0.99), indicating moderate clinical utility. Notably, 79.5% of oophorectomies were performed for lesions that were ultimately proven benign. If PRMI had been used to guide management, 26/35 (74.3%) benign oophorectomies could potentially have been avoided. The PRMI demonstrated moderate accuracy and a high negative predictive value in this cohort, supporting its potential role as an adjunctive tool in preoperative risk stratification of pediatric ovarian masses. Greater emphasis on ovary-sparing strategies is warranted to mitigate long-term reproductive and hormonal consequences in young patients.

Publisher

Springer Science and Business Media LLC

ISSN

1437-9813