MEDULLOMYOBLASTOMA

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==Definition==
 
==Definition==
 
Medullomyoblastoma is a highly malignant, bimodal embryonal tumor of the cerebellum characterized by a neuroepithelial and striated muscle component. Medullomyeloblastoma corresponds to WHO Grade IV.
 
Medullomyoblastoma is a highly malignant, bimodal embryonal tumor of the cerebellum characterized by a neuroepithelial and striated muscle component. Medullomyeloblastoma corresponds to WHO Grade IV.

Latest revision as of 18:27, 10 July 2008

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Contents

[edit] Definition

Medullomyoblastoma is a highly malignant, bimodal embryonal tumor of the cerebellum characterized by a neuroepithelial and striated muscle component. Medullomyeloblastoma corresponds to WHO Grade IV.

[edit] Epidemiology

The age distribution and clinical behavior of medullomyoblastoma mirrors those of childhood medulloblastoma, suggesting that medullomyoblastoma may be a variant of medulloblastoma.

[edit] Localization

Medullomyeloblastoma is a tumor of the cerebellar vermis.

[edit] Clinical presentation

Patients present with cerebellar signs and with signs and symptoms of raised intracranial pressure and hydrocephalus.

[edit] Imaging

On MRI, medullomyeloblastomas appear as heterogeneously enhancing, irregular mass lesions arising in the posterior fossa. Non-invasive imaging does not distinguish between medullomyeloblastoma and medulloblastoma.

[edit] Macroscopy

Medullomyeloblastomas are soft, moderately vascular tumors with variable degrees of necrosis.

[edit] Histopathology

The histopathologic appearance of medullomyeloblastoma is of a medulloblastoma with focal areas of myogenic differentiation. The myogenic component consists of bundles of striated muscle fibers, occasionally collected around tumor vessels.

[edit] Prognosis

The biologic behavior of medullomyoblatoma mirrors that of medulloblastoma.

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