Ask the Experts

Question: We are seeing a lot of information in the news about new treatments for pancreatic cancer. Is any of this information relevant to VHL? Besides, what is the cellular distinction (if any) between VHL-related pancreatic cancer and the sporadic pancreatic cancer in the general population? When we read things about prevention and cure of pancreatic cancer, how much of that is relevant to VHL? If someone with VHL has metastatic cancer from the pancreas, are these proposed therapies for pancreatic cancer likely to help? -- Confused

Answer: Most if not all of this information pertains to adenocarcinoma of the pancreas. Adenocarcinoma of the pancreas is not seen in association with VHL at any higher rate than the general population. VHL does not give an increased risk for adenocarcinoma as far as we have seen.

The type of pancreatic tumors that VHL patients get are neuroendocrine tumors of the pancreas. These arise from different types of cells than adenocarcinomas and are far less aggressive.

Neuroendocrine tumors of the pancreas can occur in patients without VHL as well, although they are very uncommon. The neuroendocrine tumors in patients with VHL tend to be very slow growing and only rarely (about 18% of patients with these tumors) do they spread to other organs like the liver. When they do spread, they can cause problems which is why we follow them very closely and remove them if they are growing or have reached a certain size.

Most, if not all of the information pertaining to adenocarcinoma of the pancreas is irrelevant for a patient with VHL neuroendocrine tumors of the pancreas.

-- Steven K. Libutti, M.D., Senior Investigator, Surgery Branch, Center for Cancer Research, National Cancer Institute, Bethesda, Maryland, USA