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Journal of Gastrointestinal Surgery

Published in:

01-06-2009 | case report

Novel Presentation of a Familial Pancreatic Cancer Syndrome

Authors: Ajay V. Maker, James A. Warth, Michael J. Zinner

Published in: Journal of Gastrointestinal Surgery | Issue 6/2009

Abstract

Introduction

Earlier detection of pancreatic cancer may help identify patients for whom surgical intervention could provide cure or prolong life. In this article, we report the occurrence of breast cancer, melanoma, squamous cell carcinoma of the alveolar ridge, colon cancer, a desmoid tumor of the abdominal wall, and pancreatic adenocarcinoma in a 65-year-old woman. She was identified as having the familial atypical multiple mole melanoma–pancreatic cancer syndrome (FAMMM–PC) with a germline p16 mutation at amino acid position 15.

Discussion

Patients with this syndrome traditionally present with multiple nevi and melanoma, and a subset also present with other cancers, including pancreatic cancer; however, no FAMMM–PC patient has yet been described with this constellation of cancers, including squamous cell carcinoma of the alveolar ridge and a desmoid tumor. Recognition of the tumors this population of patients is susceptible to developing and their genetic associations can help guide the surgeon in screening, surveillance, and eventually prevention of many of these cancers.

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Title: Novel Presentation of a Familial Pancreatic Cancer Syndrome
Authors: Ajay V. Maker
James A. Warth
Michael J. Zinner
Publication date: 01-06-2009
Publisher: Springer-Verlag
Published in: Journal of Gastrointestinal Surgery / Issue 6/2009
Print ISSN: 1091-255X
Electronic ISSN: 1873-4626
DOI: https://doi.org/10.1007/s11605-008-0780-8

At a glance: The STEP trials

Springer Medicine

Novel Presentation of a Familial Pancreatic Cancer Syndrome

Abstract

Introduction

Discussion

At a glance: The STEP trials

Springer Medicine

Abstract

Introduction

Discussion

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