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Medicine General 18b775ae

All of the following may be associated with Von Hippel Lindau syndrome, except:

A
Retinal and cerebellar hemangioblastomas
B
Gastric carcinoma
C
Pheochromocytoma
D
Renal cell carcinoma
High-Yield Explanation
Von Hippel-Lindau disease (VHL) is associated with the development of retinal angiomas, central nervous system hemangioblastomas, pheochromocytomas, multicentric clear cell cysts, hemangiomas, and adenomas of the kidney. Half of the patients with renal cysts develop clear cell carcinoma. VHL is a rare autosomal dominant disease characterized by abnormal angiogenesis with benign and malignant tumors affecting multiple tissues. It is inherited as a mutation in one allele of the VHL tumor-suppressor gene. Ref: Harrison's Internal Medicine, 18th Edition, Chapters 284, 379

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