Von Hippel Lindau disease is most commonly associated with which of the following tumors?

Prepare for USMLE Step 1 Pathology Exam with comprehensive quizzes, flashcards, and detailed explanations. Enhance your understanding and be exam-ready!

Von Hippel-Lindau (VHL) disease is a genetic disorder that predisposes individuals to various types of tumors, notably hemangioblastomas and renal cell carcinoma. The most characteristic and classic association in VHL disease is with cerebellar hemangioblastomas, which are vascular tumors that commonly occur in the cerebellum. These tumors typically arise in young adults and are often found alongside other central nervous system lesions associated with VHL.

Cerebellar hemangioblastomas are part of the broader spectrum of neoplasms seen in VHL, which also includes retinal hemangiomas, pancreatic neoplasms, and renal cell carcinoma. However, the presence of cerebellar hemangioblastomas is a hallmark finding in VHL, often leading to neurological symptoms due to brain compression or associated hydrocephalus.

While renal adenocarcinoma is also associated with VHL disease, it is not the most commonly recognized tumor in the context of VHL. The other options like osteosarcoma and neuroblastoma are not typically linked with VHL disease and are more associated with a different set of genetic and environmental factors. Thus, cerebellar hemangioblastoma is properly identified as the tumor most commonly associated with Von

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