PMID- 10067927 OWN - NLM STAT- MEDLINE DCOM- 19990401 LR - 20041117 IS - 0022-3085 (Print) IS - 0022-3085 (Linking) VI - 90 IP - 3 DP - 1999 Mar TI - Primary cerebellar glioblastomas multiforme in children. Report of four cases. PG - 546-50 AB - Primary cerebellar glioblastomas multiforme are exceedingly rare in children. The authors therefore retrospectively characterized the clinical behavior and pathological features of these tumors. A review of the database at the Hospital for Sick Children, Toronto, Canada revealed four patients with cerebellar tumors that displayed significant pleomorphism, hypercellularity, mitoses, and necrosis with pseudopalisading. The authors performed a detailed clinical, radiological, histological, and immunohistochemical analysis of the tumors in these four children (three boys and one girl; average age at presentation 7 years; range 21 months-15 years). Magnetic resonance imaging and computerized tomography most commonly revealed a large lesion with minimal edema, inhomogeneous contrast enhancement, and a discrete border. Tumor resection was subtotal in one patient and gross total in three patients. Immunostaining of the tumor cells with antisera to glial fibrillary acidic protein and vimentin was positive in varying degrees. Initial adjuvant therapy consisted of local radiation only (one patient), chemotherapy only (one patient), and radiation and chemotherapy (one patient). One patient received no adjuvant therapy. Tumor recurrence was documented in all patients: two local recurrences (at 3.5 and 7 months), one spinal recurrence (at 14 months), and one local recurrence with ventricular and spinal spread (at 8 months). Ultimately, three of the four patients developed leptomeningeal tumor spread. Patient follow up ranged from 8 to 17 months (mean 12.5 months). Three patients were dead at last follow up with a mean survival of 15 months. The prognosis for patients with cerebellar glioblastomas is extremely poor, and the tumor has a tendency for cerebrospinal fluid dissemination. The optimal management of patients harboring of these difficult-to-treat tumors, including the role of craniospinal radiation and chemotherapy, has not yet been achieved. FAU - Kulkarni, A V AU - Kulkarni AV AD - Division of Neurosurgery, Hospital for Sick Children, University of Toronto, Ontario, Canada. FAU - Becker, L E AU - Becker LE FAU - Jay, V AU - Jay V FAU - Armstrong, D C AU - Armstrong DC FAU - Drake, J M AU - Drake JM LA - eng PT - Journal Article PL - United States TA - J Neurosurg JT - Journal of neurosurgery JID - 0253357 SB - IM MH - Adolescent MH - Cerebellar Neoplasms/diagnosis/*surgery MH - Child MH - Combined Modality Therapy MH - Female MH - Glioblastoma/diagnosis/*surgery MH - Humans MH - Infant MH - Magnetic Resonance Imaging MH - Male MH - Neoplasm Recurrence, Local/therapy MH - Survival Analysis MH - Treatment Failure EDAT- 1999/03/06 00:00 MHDA- 1999/03/06 00:01 CRDT- 1999/03/06 00:00 PHST- 1999/03/06 00:00 [pubmed] PHST- 1999/03/06 00:01 [medline] PHST- 1999/03/06 00:00 [entrez] AID - 10.3171/jns.1999.90.3.0546 [doi] PST - ppublish SO - J Neurosurg. 1999 Mar;90(3):546-50. doi: 10.3171/jns.1999.90.3.0546.