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Malignant Rhabdoid Tumour of the Pineal Region

Published online by Cambridge University Press:  18 September 2015

Matthew Muller
Affiliation:
Brain Tumour Research Laboratory, University of Toronto, Toronto
Sherri Lynn Hubbard
Affiliation:
Brain Tumour Research Laboratory, University of Toronto, Toronto
John Provias
Affiliation:
Divisions of Neuropathology
Mark Greenberg
Affiliation:
Department of Oncology, Hospital for Sick Children, Toronto
Laurence E. Becker
Affiliation:
Divisions of Neuropathology
James T. Rutka
Affiliation:
Divisions of Neurosurgery
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Abstract:

A 9-month-old male presented to hospital with signs and symptoms of raised intracranial pressure. A CT scan showed obstructive hydrocephalus from a large pineal region mass lesion into which an intratumoral hemorrhage had occurred. A posterior fossa craniectomy and subtotal excision of the mass lesion were performed. By histopathology, the lesion was a malignant rhabdoid tumour (MRT). Despite surgery and chemotherapy, the tumour grew inexorably, and the patient died four months after the initial diagnosis. MRT is a rare and highly invasive neoplasm which infrequently arises from the central nervous system. This is the first documented case of a MRT arising from the pineal region. The clinical, radiographic, and pathological features of the MRT in this patient are presented.

Type
Original Articles
Copyright
Copyright © Canadian Neurological Sciences Federation 1994

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