Background: 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.
Methods: All female patients requiring operation for ovarian tumours were identified during the study period 1990-2018. Patient demographics, clinical presentation, and management are highlighted.
Results: 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).
Conclusions: 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.
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http://dx.doi.org/10.1007/s00383-021-04950-x | DOI Listing |
J Endocr Soc
January 2025
Division of Pediatric Endocrinology, Hadassah Medical Center, Jerusalem 91240, Israel.
Context: Despite a growing number of studies, the genetic etiology in many cases of ovarian dysgenesis is incompletely understood.
Objectives: This work aimed to study the genetic etiology causing absence of spontaneous pubertal development, hypergonadotropic hypogonadism, and primary amenorrhea in 2 sisters.
Methods: Whole-exome sequencing was performed on DNA extracted from peripheral lymphocytes of 2 Palestinian sisters born to consanguineous parents.
BMC Endocr Disord
January 2025
Faculty of Medicine and Health Sciences, Department of Obstetrics and Gynaecology, University of Zimbabwe, P. O. Box A178, Avondale, Harare, Zimbabwe.
Background: Proper planning of reproductive health needs for HIV-infected adolescents requires a clear understanding of the effects of HIV infection on adolescents' pubertal development.
Objective: To assess the effects of HIV infection on the hypothalamic-pituitary-ovarian (HPO) axis, ovarian reserve and pubertal development in adolescent girls at a tertiary hospital in Zimbabwe.
Methods: This was a cross-sectional survey of HIV-infected adolescent girls aged 10-19 years, with available CD4 + count results at a tertiary hospital in Zimbabwe.
Front Endocrinol (Lausanne)
January 2025
Department of Obstetrics and Gynecology, The Seventh Medical Center of Chinese People's Liberation Army (PLA) General Hospital, Beijing, China.
Introduction: Premature ovarian insufficiency (POI) is a condition characterized by ovarian dysfunction occurring before the age of 40, and its etiology is multifactorial, including genetic, immunological, infectious, environmental, and iatrogenic factors, with over half of the cases remaining unexplained. Whether the microbial communities and metabolites in follicular fluid, which is the direct microenvironment for oocyte survival, are related to POI has not been reported.
Methods: In this study, Follicular fluid samples of 26 patients with POI and 27 controls with a normal ovarian reserve were collected and analyzed using 16S rDNA sequencing and untargeted metabolomics.
Signal Transduct Target Ther
January 2025
Cluster of Excellence iFIT (EXC2180) "Image-guided and Functionally Instructed Tumor Therapies" University of Tübingen, Tübingen, Germany.
Cancer Causes Control
January 2025
Office of Cancer Survivorship, Division of Cancer Control and Population Sciences, National Cancer Institute, Bethesda, MD, USA.
Purpose: Rare cancers are defined as those for which there are less than 15 cases per 100,000 in the population annually. While much progress in detection and treatment has been made over the past decade for many rare cancers, less progress has been made in understanding survivorship needs. The objective of this study was to characterize the National Institutes of Health (NIH) cancer survivorship grant portfolio focused on rare cancers and to identify gaps specific to this area of science.
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