Which option represents an extrarenal manifestation of VHL involving the pancreas?

Which option represents an extrarenal manifestation of VHL involving the pancreas?

• A. Cavernous sinus thrombosis
• B. Adrenal cortical adenoma
• C. Splenic infarct
• D. Pancreatic cysts

Answer: (D)

Pancreatic cysts are a recognized extrarenal manifestation of VHL, reflecting the disease’s tendency to form cystic lesions beyond the kidneys. In VHL, the pancreas commonly harbors simple cysts or serous cystadenomas, which can be multiple and may be found incidentally. This pattern of pancreatic involvement is well established and fits with how VHL affects other organs through cystic or cystic-neoplastic lesions. The other options don’t fit the pancreatic extrarenal pattern seen in VHL. Cavernous sinus thrombosis isn’t a typical VHL manifestation, whereas VHL more classically involves CNS and retinal hemangioblastomas. Adrenal cortical adenoma is not a VHL hallmark; VHL is associated with pheochromocytoma arising from the adrenal medulla, not cortical adenomas. Splenic infarcts are not characteristic of VHL either. Thus, pancreatic cysts best represent the pancreatic extrarenal involvement in VHL.

Pancreatic cysts are a recognized extrarenal manifestation of VHL, reflecting the disease’s tendency to form cystic lesions beyond the kidneys. In VHL, the pancreas commonly harbors simple cysts or serous cystadenomas, which can be multiple and may be found incidentally. This pattern of pancreatic involvement is well established and fits with how VHL affects other organs through cystic or cystic-neoplastic lesions.

The other options don’t fit the pancreatic extrarenal pattern seen in VHL. Cavernous sinus thrombosis isn’t a typical VHL manifestation, whereas VHL more classically involves CNS and retinal hemangioblastomas. Adrenal cortical adenoma is not a VHL hallmark; VHL is associated with pheochromocytoma arising from the adrenal medulla, not cortical adenomas. Splenic infarcts are not characteristic of VHL either. Thus, pancreatic cysts best represent the pancreatic extrarenal involvement in VHL.