Familial gastric type 1 neuroendocrine tumor

Familial gastric type 1 neuroendocrine tumor is a rare inherited condition in which small, slow-growing tumors develop in the lining of the stomach. These tumors arise from special hormone-producing cells called enterochromaffin-like (ECL) cells. In the familial form, the condition runs in families and is linked to an underlying tendency to develop autoimmune gastritis, a condition where the immune system attacks the stomach lining. This leads to a loss of acid-producing cells, which causes the body to produce excess amounts of a hormone called gastrin. The high gastrin levels stimulate the ECL cells to grow abnormally, eventually forming small tumors (also called gastric carcinoids). Many people with this condition have no symptoms at all, and the tumors are often found during an upper endoscopy done for other reasons. When symptoms do occur, they may include stomach discomfort, nausea, or signs of vitamin B12 deficiency such as fatigue and anemia. In rare cases, the tumors may cause flushing or other symptoms related to hormone release. Type 1 gastric neuroendocrine tumors are generally considered low-grade and have a good prognosis. Treatment typically involves regular monitoring with endoscopy and removal of polyps when found. In most cases, these tumors do not spread to other parts of the body. However, because this is a familial condition, family members may also need screening. Treatment options range from endoscopic removal of tumors to medications that reduce gastrin levels, and in rare advanced cases, surgery may be considered.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Bring these to your next appointment

• Q1.How often will I need an endoscopy, and what will you be looking for each time?,Should my family members be screened for this condition, and if so, how?,Do I need genetic testing, and what would the results mean for my family?,What are the signs that a tumor might be becoming more aggressive?,Will I need vitamin B12 injections for the rest of my life?,Are there any medications that can help prevent new tumors from forming?,What lifestyle changes or dietary adjustments should I make?

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