Hemangioblastoma associated with VHL are most common in?
ANSWER: (A) CerebellumREF: Robbins 8th ed ch: 28Von Hippel-Lindau Disease:This is an autosomal dominant disease in which affected individuals develop hamangioblastomas and cysts involving the pancreas, liver, and kidneys, and have a propensity to develop renal cell carcinoma and pheochromocytoma. Hamangioblastomas are most common in the cerebellum and retinaThe gene associated with von Hippel-Lindau disease (VHL), a tumor suppressor gene, is located on chromosome 3p25-p26. It is the dysregulation of erythropoietin that is responsible for the polycythemia observed in association with hamangioblastomas in about 10% of cases.Hamangioblastomas are highly vascular neoplasms that occur as a mural nodule as sociated with a large fluid-filled cyst. On microscopic examination, the lesion consists of a mixture of variable proportions of capillary-size or somewhat larger thin-walled vessels with intervening stromal cells of uncertain histogenesis characterized by vacuolated, lightly PAS-positive, lipid-rich cytoplasm and an indefinite immunohistochemical phenotype; nonetheless, studies have shown that these cells are the neoplastic element of the hamangioblastomas based on the presence of a second "hit" in the previously normal VHL allele.
Cerebellum
Liver
Kidneys
Pancreas
Tumors of Nervous System