CASE REPORT

Intraductal papillary mucinous neoplasm of the pancreas in a patient with attenuated familial adenomatous polyposis

R Chetty¹, S Salahshor², B Bapat⁴, T Berk³, M Croitoru⁴ and S Gallinger⁵

¹ Department of Pathology, Department of Laboratory Medicine and Pathobiology, University of Toronto and University Health Network, Toronto, ON M5G 2M9, Canada
² Department of Medical Biophysics, Ontario Cancer Institute
³ Familial GI Cancer Registry, Samuel Lunenfeld Research Institute, Mount Sinai Hospital, Toronto, Ontario M5G 1X5, Canada
⁴ Center for Cancer Genetics, Samuel Lunenfeld Research Institute, Mount Sinai Hospital
⁵ Department of Surgery, University of Toronto and University Health Network, Toronto, Canada

Correspondence to:
Correspondence to:
Dr R Chetty
University Health Network, Princess Margaret Hospital, 610 University Avenue, Fourth Floor, Suite 302, Room 312, Toronto, ON M5G 2M9, Canada; runjan.chetty{at}uhn.on.ca

A 67 year old man with a clinical diagnosis of attenuated familial adenomatous polyposis (AFAP) and a past history of synchronous colon cancers in the transverse colon was also found to have an intraductal papillary mucinous neoplasm (IPMN) of the pancreas. In addition, several foci of heterotopic gastric oxyntic mucosa were noted in the duodenum, interspersed with flat and polypoid adenomas. The duodenal adenomas showed low grade dysplasia, loss of adenomatous polyposis coli (APC) protein expression, but retention of ß catenin staining, localised to the nucleus and cytoplasm. The IPMN in the pancreas showed an identical immunohistochemical profile to the duodenal adenomas. The heterotopic gastric foci in the duodenum were negative for the APC protein, and ß catenin staining was membranous in location. Although the patient did not show germline truncating APC mutations or mutations in the MYH gene, the past history, clinical features, and immunohistochemical profile of the various lesions suggest strongly that the IPMN is part of the spectrum of lesions encountered in AFAP. Whether the heterotopic oxyntic gastric mucosa in the duodenum is also related is unclear, but it may represent a forme fruste of fundic gland polyps.

Abbreviations: AFAP, attenuated familial adenomatous polyposis; APC, adenomatous polyposis coli; FAP, familial adenomatous polyposis; IPMN, intraductal papillary mucinous neoplasm

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