Features of supratentorial primitive neuroectodermal tumors in young children

Abstract

Introduction. Supratentorial primitive neuroectodermal tumors (sPNET) are highly malignant neoplasms with much more worse prognosis than at medulloblastomas. A retrospective analysis of treatment in the clinic of children under 3 years with sPNETs are given.

Materials and methods. In the period from 1998 and 2009 years, 20 children under 3 years with sPNET, there were 10 boys and 10 girls have been treated in the clinic — 4.6% of all infants with brain tumors, diagnosed for that period of time.

Results. Total tumor resection was performed in 6 (30%) patients, subtotal — in 6 (30%), partial — in 6 (30%), in 2 — biopsy was made. Postoperative lethality — 22%. In 9 (45%) observations the tumor was classified as cerebral neuroblastoma, in 1 (5%) — as cerebral ganglioneuroblastoma. Tumor cells in liquor were found in 11.8% cases, metastasis in other parts of the brain — in 15%, extraneural dissemination — in 2 patients. 11 (73.3%) underwent chemotherapy, 3 (20%) — radiation therapy. Follow-up data from 1 year to 7 years are available in 86.7% patients. The median survival was 42 months, 2- and 5-year survival — 61.5 and 15.4% respectively, recurrence rate — 20%.

Conclusions. sPNET in children are relatively rare, they are one of the most malignant brain tumors. Chemotherapy inclusion into complex treatment promotes survival prolongation. To improve results of treatment in children with sPNET further search for effective complex of combined treatment of such tumors is required, especially in young children.