Which statement best identifies a syndrome associated with pancreatic cysts and visceral cysts including those in the pancreas?

Which statement best identifies a syndrome associated with pancreatic cysts and visceral cysts including those in the pancreas?

• A. Von Hippel-Lindau syndrome
• B. Turner syndrome
• C. Ehlers-Danlos syndrome
• D. Klinefelter syndrome

Answer: (A)

Von Hippel-Lindau syndrome is a hereditary condition where cysts and tumors appear in multiple organs, including the pancreas. It’s caused by mutations in the VHL gene and is usually inherited in an autosomal dominant pattern. Pancreatic involvement often includes cysts or serous cystadenomas, and there can be visceral cysts in organs like the kidneys and liver, along with other hallmark findings such as CNS hemangioblastomas and retinal angiomas. Because pancreatic cysts with visceral cysts are characteristic of this syndrome, it’s the best fit among the options. The other listed syndromes have different typical features and are not associated with pancreatic or visceral cysts.

Von Hippel-Lindau syndrome is a hereditary condition where cysts and tumors appear in multiple organs, including the pancreas. It’s caused by mutations in the VHL gene and is usually inherited in an autosomal dominant pattern. Pancreatic involvement often includes cysts or serous cystadenomas, and there can be visceral cysts in organs like the kidneys and liver, along with other hallmark findings such as CNS hemangioblastomas and retinal angiomas. Because pancreatic cysts with visceral cysts are characteristic of this syndrome, it’s the best fit among the options. The other listed syndromes have different typical features and are not associated with pancreatic or visceral cysts.