TREATMENT OF NONGERMINOMATOUS GERM-CELL TUMORS OF THE PINEAL REGION

GERM-CELL TUMORS CAN be subdivided into germinoma, embryonal carcinoma , choriocarcinoma, endodermal sinus tumor (yolk-sac tumor), and terato ma. They are also distinguished by their production of secreted marker s such as alpha-fetoprotein produced in endodermal sinus tumors and em bryonal carcinoma or beta-human chorionic gonadotropin, produced by ch oriocarcinoma and embryonal carcinoma. Germinoma and teratoma produce none of the markers. Because it has been proposed that teratomas may d ifferentiate from multipotent stem cells contained in embryonal carcin oma and are thus lineage related, the presence of markers indicates th e presence of a nongerminomatous germ-cell tumor. Nongerminomatous ger m-cell tumors are an invariably fatal subgroup within the pediatric pi neal region germ-cell tumors. There is no effective, established thera peutic regimen. We report the treatment regimen for three children dia gnosed with this highly aggressive tumor entity. The children were fir st given a course of chemotherapy with bleomycin, etoposide, and cispl atin. This resulted in the normalization of markers and the shrinkage of tumors. These were then removed by the infratentorial supracerebell ar approach. Removal was followed by a second course of chemotherapy w ith vinblastine, ifosfamide, and cisplatin; after which the children u nderwent radiotherapy. All three children are well and without evidenc e of residual or recurrent disease 20, 30, and 32 months after surgery , respectively. We propose this therapy regimen for children in whom t he markers are positive.