Supratentorial embryonal tumors in children under 5 years of age: An SFOP study of treatment with postoperative chemotherapy alone

Background To determine the effectiveness of multiagent chemotherapy as sole postoperative treatment of supratentorial central nervous system (CNS) embryonal tumors in young children. Procedure. The data of 25 children under 5 years of age diagnosed with supratentorial embryonal tumors (17 primitive neuroectodermal tumors, four pinealoblastomas, and four medulloepitheliomas) treated exclusively by postoperative chemotherapy (CT) between 1990 and 1997 were reviewed. Results. Fifteen tumors were hemispheric and 10 were deeply seated. Four children presented with disseminated leptomeningeal disease. Total resection was performed in nine patients, subtotal in 9, partial in 3, and a diagnostic biopsy only in 2. Two children did not undergo surgery. Twenty-four children relapsed with a median time of 5.5 months. The median overall survival was 12 months, and the 2-, and 5- year survivals were 30 and 14%, respectively. The 2-year disease-free survival was 4%. There was a significantly worse prognosis in patients undergoing incomplete resection and in the group with deeply situated tumors. Four relapses were treated by second surgery followed by highdose chemotherapy and radiotherapy. Two of them remain in CR2, and all these children are free of late sequelae. Conclusions. CT alone failed to maintain disease-free survival in most of the children, although, disease progression was delayed to some extent. Children under 5 years with supratentorial embryonal tumors should undergo total surgical resection if possible. (C) 2002 Wiley-Liss, Inc.