Endodermal sinus tumor and embryonal carcinoma of the ovary in children

Abstract

The clinicopathological features of 26 pure endodermal sinus tumors (EST) and for embryonal carcinomas of the ovary occurring in children under age 15 years in Britain over the period 1962–1978 are described. Endodermal sinus tumor has shown a significant increase (P < 0.001) over the 17-year period considered. Compared to controls, EST and embryonal carcinoma have not shown an association with the use of hormones or tranquilizers during pregnancy, with radiological investigations, or with infections in pregnancy though the numbers of cases studied were very small. The histological and immunohistological features of EST and embryonal carcinoma in children do not differ substantially from the descriptions of these tumors in adolescents and adults. Using the FIGO staging classification 23% were Stage I tumors; 3% were Stage II; 70% were Stage III; and 3% were Stage IV neoplasms. The 5-year actuarial survival rate was 39% among 26 cases of EST and 1 of the 4 cases of embryonal carcinoma also survived for 5 years. Clinical stage at diagnosis was the only indicator of prognosis, but the introduction of polychemotherapy (vincristine, actinomicin-D, cyclophosphamide/ cis-platinum, vincristine, bleomycin) resulted in long-term survival in 7 of 7 cases of EST (2 Stage I and 5 Stage III) and 1 of 2 cases of embryonal carcinoma (Stage III). Radiotherapy appears to have little role to play in the treatment of pure EST or embryonal carcinoma.