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What is VHL syndrome is associated most commonly with which carcinoma - and explain in detail?
Von Hippel-Lindau (VHL) syndrome: VHL gene is a tumor suppressor gene and is involved in the development of both familial and sporadic clear cell carcinoma. VHL patients develop bilateral renal cysts and multiple renal cell carcinomas. Both sporadic and familial RCC is associated with: Loss of sequence on chromosome 3 either by translocation (3:6, 3:8, 3:11) or deletion. This region harbors the VHL gene. Familial RCC is associated with:- Von Hippel-Lindau syndrome VHL syndrome: Autosomal dominant cancer syndrome, characterized by: Cerebellar hemangioblastomas Retinal angiomas Clear cell RCC Pheochromocytoma Cysts in various organs. Ref: RAM DAS NAYAK EXAM PREPARATORY MANUAL FOR UNDERGRADUATES 2nd ed. pg no: 633