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| Hemangioblastoma | ||
| Etiology •Can occur sporadically or in Von Hippel-Lindau's disease where they are multiple. | ||
| Pathogenesis •See etiology., | ||
| 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. | ||
| General Gross Description •Hemangioblastomas are circumscribed but not encapsulated tumors which appear as reddish blue lesions often associated with a cyst. •Examples: | ||
| 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. •Examples: | ||
| 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. | ||
| 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. Please be patient during transfer. Medline will open in a new window. To return, close the Medline Window Hemangioblastoma
| Synopsis by: M. L. Grunnet M.D. (TX6000M91611)[411]
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