Hereditary neuroendocrine tumor of small intestine

Hereditary neuroendocrine tumor of the small intestine is a rare inherited form of cancer that develops in the neuroendocrine cells lining the small bowel. Neuroendocrine cells are special cells that receive signals from the nervous system and respond by releasing hormones into the blood. When these cells grow out of control, they form tumors sometimes called carcinoid tumors. In the hereditary form, a genetic predisposition runs in families, meaning that affected individuals carry gene changes that increase their risk of developing these tumors compared to the general population. Symptoms often develop slowly and may not appear until the disease is advanced. Common signs include abdominal pain, diarrhea, flushing of the skin, and wheezing. When the tumor produces excess hormones such as serotonin, patients may develop what is called carcinoid syndrome, which causes episodes of facial flushing, diarrhea, and sometimes heart valve problems. Some patients may also experience bowel obstruction if the tumor grows large enough to block the intestine. Treatment depends on the stage of the disease and may include surgery to remove the tumor, somatostatin analogs (such as octreotide or lanreotide) to control hormone-related symptoms, targeted therapies like everolimus, and peptide receptor radionuclide therapy (PRRT). Because this is a hereditary form, family members may benefit from genetic counseling and screening to detect tumors early, when they are most treatable.

Bring these to your next appointment

• Q1.What stage is my tumor, and has it spread beyond the small intestine?,Is surgery an option for me, and could it be curative?,Which genetic test should I and my family members have, and what do the results mean?,What screening schedule do you recommend for my at-risk relatives?,What are the side effects of somatostatin analog therapy, and how will we manage them?,Should I be monitored for carcinoid heart disease, and how often?,Are there any clinical trials available for hereditary neuroendocrine tumors that I might be eligible for?

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