This is a real-world medical entrance exam question, please give the true answer based on the question and selection. Topic name: Urinary tract, Subject name: Pathology
Bilateral Renal cell carcimoma is seen in 
Select and explain. 
A. Eagle Barett's syndrome, B. Beckwith Weideman syndrome, C. Von Hippel Lindau disease, D. Bilateral Angiomyolipoma
C: Von Hippel Lindau disease
Answer: C: Von Hippel Lindau disease 
Explanation: VHL syndrome: Autosomal dominant cancer syndrome, characterized by: Cerebellar hemangioblastomas Retinal angiomas Clear cell RCC Pheochromocytoma Cysts in various organs. Patients of VHL disease have germline mutations of tumor suppressor VHL gene located on chromosome 3p, commonly as homozygous loss of the VHL gene. About 35% of cases of VHL develop RCC. Ref: Ram Das Nayak 2nd edition page no: 633