Multiple endocrine neoplasia type 1 natural history, complications and prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ammu Susheela, M.D. [2]
Overview
The natural history largely depends on the manifestation and virulence of the manifestations. The manifestations of multiple endocrine neoplasia type 1 usually develop in the first, second, or third decade of life. If left untreated, manifestations of multiple endocrine neoplasia type 1 may gradually worsen, and patients may die of complications of the disease. Life-threatening complications of multiple endocrine neoplasia type 1 include gastrointestinal bleeding, convulsions, hypokalemia, hypoglycemia, and venous thrombosis. Although many tumors associated with multiple endocrine neoplasia type 1 are benign, approximately half of people with multiple endocrine neoplasia type 1 eventually develop a cancerous tumor. The prognosis of multiple endocrine neoplasia type 1 is generally good with treatment. Development of pancreatic cancer is associated with poor prognosis.
Natural History
- The natural history of multiple endocrine neoplasia type 1 is difficult to delineate given the rarity of the disease. The natural history largely depends on the manifestation and virulence of the manifestations.
- The manifestations of multiple endocrine neoplasia type 1 usually develop in the first, second, or third decade of life.
- The age at which multiple endocrine neoplasia type 1 develops may vary significantly, even among members of the same family.
- If left untreated, manifestations of multiple endocrine neoplasia type 1 may gradually worsen, and patients may die of complications of the disease.
Complications
- Complications that can develop as a result of multiple endocrine neoplasia type 1 are as below.[1]
Prognosis
- The prognosis of multiple endocrine neoplasia type 1 is generally good with treatment.
- Early death can occur due to the effect of hormones produced by these tumors.
- Although many tumors associated with multiple endocrine neoplasia type 1 are benign, approximately half of people with multiple endocrine neoplasia type 1 will eventually develop a cancerous tumor.
- The presence of pancreatic tumors is associated with a particularly poor prognosis among patients with multiple endocrine neoplasia type 1.
References
- ↑ Falchetti A, Marini F, Luzi E, Giusti F, Cavalli L, Cavalli T; et al. (2009). "Multiple endocrine neoplasia type 1 (MEN1): not only inherited endocrine tumors". Genet Med. 11 (12): 825–35. doi:10.1097/GIM.0b013e3181be5c97. PMID 19904212.