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Pancreas

Pancreatic endocrine neoplasms

Tumors in MEN1 patients


Reviewer: Deepali Jain, M.D. (see Reviewers page)
Revised: 4 December 2012, last major update August 2012
Copyright: (c) 2001-2012, PathologyOutlines.com, Inc.

General
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● Pancreatic endocrine tumors can occur in 4 inherited disorders: Multiple Endocrine Neoplasia type 1 (MEN1), von Hippel-Lindau disease (VHL), neurofibromatosis 1 (NF1) (von Recklinghausen disease), tuberous sclerosis complex (TSC) (Cancer 2008;113:1807) - discussion below focuses on MEN1
● Usually produce glucagon, insulin or pancreatic polypeptide
● Tendency towards multiple tumors in same patient, often microscopic, which often produce different hormones
● Associated with nesidioblastosis in 30% (Am J Surg Pathol 1996;20:1378)
● Loss of ATRX (alpha thalassemia/mental retardation X-linked) and DAXX (death domain-associated protein) tumor suppressor genes with alternative lengthening of telomeres is a late event in pancreatic neuroendocrine tumors associated with MEN1 syndrome (Mod Pathol 2012;25:1033, Science 2011;331:1199)
● Mutations in MEN1 and DAXX/ATRX genes associated with better prognosis
● Mutations in genes in the mammalian target of rapamycin (mTOR) pathway found in 14% of tumors

Diagrams
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Location of tumors

End of Pancreas > Pancreatic endocrine neoplasms > Tumors in MEN1 patients


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