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==Complications== | ==Complications== | ||
* Complications that can develop as a result of MEN-1 are as follows.<ref name="pmid19904212">{{cite journal| author=Falchetti A, Marini F, Luzi E, Giusti F, Cavalli L, Cavalli T et al.| title=Multiple endocrine neoplasia type 1 (MEN1): not only inherited endocrine tumors. | journal=Genet Med | year= 2009 | volume= 11 | issue= 12 | pages= 825-35 | pmid=19904212 | doi=10.1097/GIM.0b013e3181be5c97 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19904212 }} </ref> <ref name="pmid27811505">{{cite journal| author=Nell S, Borel Rinkes IH, Verkooijen HM, Bonsing BA, van Eijck CH, van Goor H et al.| title=Early and Late Complications After Surgery For MEN1-related Nonfunctioning Pancreatic Neuroendocrine Tumors. | journal=Ann Surg | year= 2016 | volume= | issue= | pages= | pmid=27811505 | doi=10.1097/SLA.0000000000002050 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27811505 }} </ref> <ref name="pmid27247513">{{cite journal| author=Kwon EB, Jeong HR, Shim YS, Lee HS, Hwang JS| title=Multiple Endocrine Neoplasia Type 1 Presenting as Hypoglycemia due to Insulinoma. | journal=J Korean Med Sci | year= 2016 | volume= 31 | issue= 6 | pages= 1003-6 | pmid=27247513 | doi=10.3346/jkms.2016.31.6.1003 | pmc=4853657 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27247513 }} </ref> <ref name="pmid23569534">{{cite journal| author=Li J, Zeng L, Yang Y, Zhan Y, Tao J, Wu B| title=Multiple endocrine neoplasia type 1- presenting multiple lipomas and hypoglycemia onset. | journal=Am J Case Rep | year= 2012 | volume= 13 | issue= | pages= 224-9 | pmid=23569534 | doi=10.12659/AJCR.883383 | pmc=3616096 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23569534 }} </ref> | * Complications that can develop as a result of MEN-1 are as follows.<ref name="pmid19904212">{{cite journal| author=Falchetti A, Marini F, Luzi E, Giusti F, Cavalli L, Cavalli T et al.| title=Multiple endocrine neoplasia type 1 (MEN1): not only inherited endocrine tumors. | journal=Genet Med | year= 2009 | volume= 11 | issue= 12 | pages= 825-35 | pmid=19904212 | doi=10.1097/GIM.0b013e3181be5c97 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19904212 }} </ref> <ref name="pmid27811505">{{cite journal| author=Nell S, Borel Rinkes IH, Verkooijen HM, Bonsing BA, van Eijck CH, van Goor H et al.| title=Early and Late Complications After Surgery For MEN1-related Nonfunctioning Pancreatic Neuroendocrine Tumors. | journal=Ann Surg | year= 2016 | volume= | issue= | pages= | pmid=27811505 | doi=10.1097/SLA.0000000000002050 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27811505 }} </ref> <ref name="pmid27247513">{{cite journal| author=Kwon EB, Jeong HR, Shim YS, Lee HS, Hwang JS| title=Multiple Endocrine Neoplasia Type 1 Presenting as Hypoglycemia due to Insulinoma. | journal=J Korean Med Sci | year= 2016 | volume= 31 | issue= 6 | pages= 1003-6 | pmid=27247513 | doi=10.3346/jkms.2016.31.6.1003 | pmc=4853657 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27247513 }} </ref> <ref name="pmid23569534">{{cite journal| author=Li J, Zeng L, Yang Y, Zhan Y, Tao J, Wu B| title=Multiple endocrine neoplasia type 1- presenting multiple lipomas and hypoglycemia onset. | journal=Am J Case Rep | year= 2012 | volume= 13 | issue= | pages= 224-9 | pmid=23569534 | doi=10.12659/AJCR.883383 | pmc=3616096 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23569534 }} </ref> <ref name="pmid17168407">{{cite journal| author=Koga Y, Ohe K, Gondo S, Watanabe T, Sakamoto R, Nomura M et al.| title=[MEN type I presenting hypokalemia and hypertension, complicated with acromegaly, adrenal cortical tumor and rectal carcinoid tumor]. | journal=Nihon Naika Gakkai Zasshi | year= 2006 | volume= 95 | issue= 11 | pages= 2298-301 | pmid=17168407 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17168407 }} </ref> | ||
:* [[Necrolytic migratory erythema]] | :* [[Necrolytic migratory erythema]] | ||
:* [[Hypoglycemia]] | :* [[Hypoglycemia]] | ||
Revision as of 17:15, 6 October 2017
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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. [6]
- Although many tumors associated with MEN-1 are benign, approximately half of people with MEN-1 will eventually develop a cancerous tumor. [7]
- 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.
- ↑ 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.
- ↑ 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.