| Hemangioblastoma
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Etiology
•Can occur sporadically or in Von Hippel-Lindau's
disease where they are multiple.
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Pathogenesis
•See etiology.,
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Epidemiology
•Make approximately 1-2% of intracranial tumors and
about 7% of posterior fossa tumor.
•Occur most commonly
in the cerebellum and medulla but can be seen in the
cerebral hemispheres or spinal cord.
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General Gross Description
•Hemangioblastomas are circumscribed but not encapsulated
tumors which appear as reddish blue lesions often
associated with a cyst.
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General Microscopic Description
•Hemangioblastomas are highly vascular tumors which have
both large and capillary like vessels within them. The
capillaries predominate and are formed by endothelial
cells, pericytes and large clear foamy cell of uncertain
origin between the capillary channels. These contain
lipid which is removed during processing.
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Clinical Correlation
•The clinical signs of a hemangioblastoma depend on
where it arises from and if it is single or multiple.
•Sporadic cases are usually single whereas in Von Hippel
Lindau disease they are often multiple.
•In the cerebellum
they can cause ataxia and increased intracranial pressure
They can also hemorrhage causing a rapid increase in
intracranial pressure.
•After removal they can recur
although they are not malignant and do not metastasize.
•In Von Hippel Lindau''s disease they tend reoccur in
multiple areas for reasons that are not clear. Von
Hippel Lindau's disease is associated with retinal tumors
which have the same morphology as hemangioblastomas and
renal, adrenal and other tumors and hamartomas.
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References
• Poirer J et.al. Manual of basic neuropathology. Philadelphia: Saunders, 1990, pp.46-48.
• Cotran RS, Kumar V, Robbins SL: Robbins Pathologic Basis of Disease. 5th ed. Philadelphia, W.B. Saunders, 1994, pp. 1354.
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| Hemangioblastoma
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| Synopsis by: M. L. Grunnet M.D. (TX6000M91611)[411]
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