Primary cerebellar glioblastomas multiforme in children

Report of four cases

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✓ 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.

Article Information

Address reprint requests to: James M. Drake, M.D., F.R.C.S.(C), Division of Neurosurgery, Hospital for Sick Children, 555 University Avenue, Toronto, Ontario, M5G 1X8 Canada. email: james.drake@sickkids.on.ca.

© AANS, except where prohibited by US copyright law.

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    Case 4. Left: Axial T1-weighted MR image, after gadolinium enhancement, demonstrating a large, inhomogeneously enhancing tumor with mixed signal intensity centrally, suggestive of possible intratumoral hemorrhage. This image was obtained only 2 months after a normal plain CT scan. Right: Axial T1-weighted MR image, after gadolinium enhancement, obtained at tumor recurrence 7 months after gross-total resection and chemotherapy. This image demonstrates a large local recurrence without contrast enhancement.

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