Which tumors are commonly associated with von Hippel-Lindau syndrome?

• A. Meningiomas, pituitary adenoma, glioblastoma
• B. Hemangioblastomas, clear cell renal carcinoma, pheochromocytoma
• C. Hemangiomas in the liver, renal cysts, pheochromocytoma
• D. Retinoblastoma, osteosarcoma, pancreatic cancer

Answer: (B)

Von Hippel-Lindau syndrome is a hereditary tumor predisposition caused by loss of the VHL tumor suppressor gene, which leads to stabilization of hypoxia-inducible factors and upregulation of angiogenic factors like VEGF. This explains the tendency toward highly vascular tumors. The classic set of tumors seen in this condition includes hemangioblastomas of the central nervous system and retina, clear cell renal cell carcinoma, and pheochromocytoma. Among the options, this combination best reflects the hallmark VHL tumor spectrum. Other choices include tumors or lesions that are not characteristically linked to VHL—such as nonvascular brain tumor types, liver hemangiomas or simple renal cysts without the associated RCC, or retinoblastoma and osteosarcoma which are not typical VHL manifestations.