Management of gynecologic adnexal tumors and tumor-like lesions in children and adolescents: experience at a cancer center

Purpose: Tumors and tumor-like lesions of the uterine adnexa in children and adolescents are uncommon but may carry devastating consequences. Methods We conducted an observational retrospective cohort study, to describe patients aged 0–19 years diagnosed with tumors and tumor-like lesions of the uterine adnexa at our institution between 2000–2018. Results Eighty-nine patients with 105 adnexal lesions were included. Thirty-seven(41.6%) patients presented with benign tumors, 13(14.6%) with borderline tumors, 25(28.1%) with malignant tumors and 14(15.7%) with tumor-like lesions. Germ cell tumors(n = 45|42.9%) were the most frequent, followed by epithelial tumors(n = 30|28.6%). No significant differences were found in the age distribution of the lesions by malignant potential or histologic group. Most patients(n = 80|89.9%) were treated primarily with conservative surgery, including cystectomy(n = 25|28.1%) and unilateral oophorectomy/adnexectomy(n = 54|60.7%). Thirty-four(38.2%) underwent surgical staging (partial omentectomy and peritoneal biopsies). Twenty(22.5%) patients with borderline and malignant tumors were submitted to chemotherapy. Four(4.5%) patients with borderline or malignant tumors relapsed, one of which died from disease. Conclusion Pediatric gynecological lesions encompass a wide range of rare histological tumor subtypes, requiring evaluation by experienced pathologists. Most tumors were diagnosed at early stages, with low relapse and mortality rates. Conservative management, with fertility sparing surgery and limited use of adjuvant chemotherapy, is of utmost importance.