Ovarian germ cell tumors in children: a 20-year retrospective study in a single institution.

摘要

PURPOSE: Ovarian germ cell tumors are rare in childhood. The goal of the study is to provide information that may help guide the evaluation and surgical management of future children with ovarian tumors. METHODS: A retrospective review of patients with ovarian germ cell tumors between January 1990 and January 2010 was performed. RESULTS: 137 patients were included with a median age of 9.5 years. Teratomas were found most frequently (mature: 78, immature: 6), followed by yolk sac tumors (n = 51), dysgerminoma (n = 1) and embryonal carcinoma (n = 1). Abdominal pain (81.8%) and abdominal distension (58.4%) were the most common symptoms. Twenty-six infants were found prenatally. Twenty-one patients presented torsion of the ovary. Alpha-feto-protein levels were elevated in all pure yolk sac tumors, two immature teratomas and one embryonal carcinoma. Most patients (84) were Stage I, 16 were Stage II, 23 Stage III, and four Stage IV. All patients with mature and immature teratomas (grade 1) underwent surgery alone. Surgery + chemotherapy were conducted in 55 other patients. The surgical procedures consisted of salpingo-oophorectomy (n = 68), oophorectomy (n = 21) and ovarian-sparing tumorectomy (n = 48). Sixteen patients gave up the treatment and died. Excluding this subset, 5-year relapse-free survival and overall survival was 93.4% and 98.3%, respectively. No recurrences were observed in any patients. CONCLUSION: Ovarian germ cell tumors have an excellent prognosis. With accurate staging, complete resection, and adjuvant chemotherapy, patients should be expected to have excellent survival rates. Preservation of ovarian tissue should be considered whenever safe and feasible, however, this needs to be confirmed by studies on larger numbers of patients.