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. [1]
- 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. [9]
- Although many tumors associated with MEN-1 are benign, approximately half of people with MEN-1 will eventually develop a cancerous tumor. [10]
- The presence of pancreatic tumors is associated with a particularly poor prognosis among patients with MEN-1.
References
- ↑ Shepherd JJ (1991). "The natural history of multiple endocrine neoplasia type 1. Highly uncommon or highly unrecognized?". Arch Surg. 126 (8): 935–52. PMID 1677802.
- ↑ 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.
- ↑ Kwon EB, Jeong HR, Shim YS, Lee HS, Hwang JS (2016). "Multiple Endocrine Neoplasia Type 1 Presenting as Hypoglycemia due to Insulinoma". J Korean Med Sci. 31 (6): 1003–6. doi:10.3346/jkms.2016.31.6.1003. PMC 4853657. PMID 27247513.
- ↑ Li J, Zeng L, Yang Y, Zhan Y, Tao J, Wu B (2012). "Multiple endocrine neoplasia type 1- presenting multiple lipomas and hypoglycemia onset". Am J Case Rep. 13: 224–9. doi:10.12659/AJCR.883383. PMC 3616096. PMID 23569534.
- ↑ Koga Y, Ohe K, Gondo S, Watanabe T, Sakamoto R, Nomura M; et al. (2006). "[MEN type I presenting hypokalemia and hypertension, complicated with acromegaly, adrenal cortical tumor and rectal carcinoid tumor]". Nihon Naika Gakkai Zasshi. 95 (11): 2298–301. PMID 17168407.
- ↑ Naik C, Basu S (2017). "Peptide Receptor Radionuclide Therapy with (177)Lu-DOTATATE for Metastatic Neuroendocrine Tumor Occurring in Association with Multiple Endocrine Neoplasia Type 1 and Cushing's Syndrome". World J Nucl Med. 16 (2): 126–132. doi:10.4103/1450-1147.203068. PMC 5436318. PMID 28553179.
- ↑ Schuppe HC, Neumann NJ, Schock-Skasa G, Höppner W, Feldkamp J (1999). "Secondary infertility as early symptom in a man with multiple endocrine neoplasia-type 1". Hum Reprod. 14 (1): 252–4. PMID 10374130.
- ↑ 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.