Which of the following is an extrarenal manifestation of VHL?

• A. Renal cell carcinoma
• B. Acute pancreatitis
• C. Nephrolithiasis
• D. Pheochromocytoma

Answer: (D)

Pheochromocytoma is an adrenal tumor that arises outside the kidneys and is a known extrarenal manifestation of von Hippel-Lindau disease. VHL predisposes to tumors in several organs beyond the kidneys, including CNS and retinal hemangioblastomas, pancreatic lesions, and pheochromocytomas. Among the options, the adrenal medulla tumor fits the extrarenal pattern because it originates outside the kidney and often secretes catecholamines, producing hypertension and other symptoms. Renal cell carcinoma lies in the kidney itself, so it’s a renal manifestation. Nephrolithiasis is a urinary tract stone issue, not a tumor. Acute pancreatitis is not a characteristic extrarenal tumor associated with VHL, though pancreatic lesions can occur in VHL.