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 (MEN-1) usually develop in the first, second, or third decade of life. If left untreated, manifestations of MEN-1 may gradually worsen, and patients may die of complications of the disease. Life-threatening complications of MEN-1 include gastrointestinal bleeding, convulsions, hypokalemia, hypoglycemia, and venous thrombosis. Although many tumors associated with MEN-1 are benign, approximately half of people with MEN-1 eventually develop a cancerous tumor. The prognosis of MEN-1 is generally good with treatment. Development of pancreatic cancer is associated with poor prognosis.
Natural History
- The natural history of MEN-1 is difficult to delineate given the rarity of the disease.
- The manifestations of MEN-1 usually develop in the first, second, or third decade of life.
- The age at which MEN-1 develops may vary significantly, even among members of the same family.
- If left untreated, manifestations of MEN-1 may gradually worsen, and patients may die of complications of the disease.
Complications
Prognosis
- The prognosis of MEN-1 is generally good with treatment.
- Early death can occur due to the effect of hormones produced by these tumors. [3]
- Although many tumors associated with MEN-1 are benign, approximately half of people with MEN-1 will eventually develop a cancerous tumor. [4]
- The presence of pancreatic tumors is associated with a particularly poor prognosis among patients with MEN-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.
- ↑ Nell S, Borel Rinkes IH, Verkooijen HM, Bonsing BA, van Eijck CH, van Goor H; et al. (2016). "Early and Late Complications After Surgery For MEN1-related Nonfunctioning Pancreatic Neuroendocrine Tumors". Ann Surg. doi:10.1097/SLA.0000000000002050. PMID 27811505.
- ↑ Conemans EB, Brosens LAA, Raicu-Ionita GM, Pieterman CRC, de Herder WW, Dekkers OM; et al. (2017). "Prognostic value of WHO grade in pancreatic neuro-endocrine tumors in Multiple Endocrine Neoplasia type 1: Results from the DutchMEN1 Study Group". Pancreatology. 17 (5): 766–772. doi:10.1016/j.pan.2017.07.196. PMID 28811081.
- ↑ Ito T, Igarashi H, Uehara H, Berna MJ, Jensen RT (2013). "Causes of death and prognostic factors in multiple endocrine neoplasia type 1: a prospective study: comparison of 106 MEN1/Zollinger-Ellison syndrome patients with 1613 literature MEN1 patients with or without pancreatic endocrine tumors". Medicine (Baltimore). 92 (3): 135–81. doi:10.1097/MD.0b013e3182954af1. PMC 3727638. PMID 23645327.