A Comparison of the Morphology of Familial and Sporadic Pancreatic Cancers
Aatur D Singhi, Hiroyuki Ishida, Alison P Klein, Syed Z Ali, Zina Meriden, Ralph H Hruban. Johns Hopkins University School of Medicine, Baltimore, MD
Background: Nearly 10% of patients with pancreatic cancer report a family history of the disease. Genetic syndromes, including Peutz-Jeghers and familial breast cancer (BRCA-2) increase pancreatic cancer risk. Certain subtypes of pancreatic cancer (such as medullary carcinoma) can occur more frequently in given genetic syndromes (such as Hereditary Nonpolyposis Colorectal Cancer Syndrome). The purpose of this study was to compare the morphology of familial (FC) and sporadic (SC) pancreatic cancer, with FC defined as a pancreatic cancer that occurred in an individual with a first-degree relative who also had a diagnosis of pancreatic cancer.
Design: The National Familial Pancreas Tumor Registry (NFPTR) is a research study enrolling patients with a family history of pancreatic cancer. The NFPTR includes 1201 patients with pancreatic resections whose pathology was assessed at our center. These included 529 patients with familial pancreatic cancer and 672 apparently sporadic pancreatic cancer patients. We retrospectively reviewed all available pathology slides in a blinded fashion.
Results: Primary tumor types were classified as moderately-differentiated ductal carcinoma (FC=307, SC=357); Poorly-differentiated ductal carcinoma (FC=156, SC=238), Well-differentiated ductal carcinoma (FC=23, SC=45) mixed ductal/neuroendocrine carcinoma (FC=2); well-differentiated neuroendocrine neoplasm (FC=2, SC=2); medullary (FC=1), adenosquamous (FC=6, SC=8), colloid (FC=4, SC=11), acinar (SC=1), large duct adenocarcinoma (FC=1, SC=4), undifferentiated (FC=5, SC=4), undifferentiated with osteoclast-like giant cells (FC=1, SC=1), solid-pseudopapillary neoplasm (SC=1) and neuroendocrine (FC=2, SC=2). No statistically significant differences were noted in the primary tumor types between FC's and SC's (p=0.53, overall or only non-adenocarcinomas).
Conclusions: We report the pathology of 1201 familial pancreatic cancers. Although certain subtypes of pancreatic cancer have historically been linked with genetic syndromes, the current study illustrates no statistically significant differences between the morphologies of familial vs. sporadic pancreatic cancers among the patients in our cohort.
Tuesday, March 5, 2013 1:00 PM
Poster Session IV # 160, Tuesday Afternoon