Treatment of nongerminomatous germ-cell tumors of the pineal region

Abstract

Germ-cell tumors can be subdivided into germinoma, embryonal carcinoma, choriocarcinoma, endodermal sinus tumor (yolk-sac tumor), and teratoma. They are also distinguished by their production of secreted markers such as alpha-fetoprotein produced in endodermal sinus tumors and embryonal carcinoma or beta-human chorionic gonadotropin, produced by choriocarcinoma and embryonal carcinoma. Germinoma and teratoma produce none of the markers. Because it has been proposed that teratomas may differentiate from multipotent stem cells contained in embryonal carcinoma and are thus lineage related, the presence of markers indicates the presence of a nongerminomatous germ-cell tumor. Nongerminomatous germ-cell tumors are an invariably fatal subgroup within the pediatric pineal region germ-cell tumors. There is no effective, established therapeutic regimen. We report the treatment regimen for three children diagnosed with this highly aggressive tumor entity. The children were first given a course of chemotherapy with bleomycin, etoposide, and cisplatin. This resulted in the normalization of markers and the shrinkage of tumors. These were then removed by the infratentorial supracerebellar approach. Removal was followed by a second course of chemotherapy with vinblastine, ifosfamide, and cisplatin; after which the children underwent radiotherapy. All three children are well and without evidence of residual or recurrent disease 20, 30, and 32 months after surgery, respectively. We propose this therapy regimen for children in whom the markers are positive.