Clinical and pathologic features of familial pancreatic cancer

Humphris, Jeremy L., Johns, Amber L., Simpson, Skye H., Cowley, Mark J., Pajic, Marina, Chang, David K., Nagrial, Adnan M., Chin, Venessa T., Chantrill, Lorraine A., Pinese, Mark, Mead, R. Scott, Gill, Anthony J., Samra, Jaswinder S., Kench, James G., Musgrove, Elizabeth A., Tucker, Katherine M., Spigelman, Allan D., Waddell, Nic, Grimmond, Sean M., Biankin, Andrew V. and The Australian Pancreatic Cancer Genome Inititiative (2014) Clinical and pathologic features of familial pancreatic cancer. Cancer, 120 23: 3669-3675. doi:10.1002/cncr.28863


Author Humphris, Jeremy L.
Johns, Amber L.
Simpson, Skye H.
Cowley, Mark J.
Pajic, Marina
Chang, David K.
Nagrial, Adnan M.
Chin, Venessa T.
Chantrill, Lorraine A.
Pinese, Mark
Mead, R. Scott
Gill, Anthony J.
Samra, Jaswinder S.
Kench, James G.
Musgrove, Elizabeth A.
Tucker, Katherine M.
Spigelman, Allan D.
Waddell, Nic
Grimmond, Sean M.
Biankin, Andrew V.
The Australian Pancreatic Cancer Genome Inititiative
Title Clinical and pathologic features of familial pancreatic cancer
Journal name Cancer   Check publisher's open access policy
ISSN 1097-0142
0008-543X
Publication date 2014-12-01
Year available 2014
Sub-type Article (original research)
DOI 10.1002/cncr.28863
Open Access Status DOI
Volume 120
Issue 23
Start page 3669
End page 3675
Total pages 7
Place of publication Hoboken, NJ, United States
Publisher John Wiley & Sons
Collection year 2015
Language eng
Formatted abstract
BACKGROUND: Inherited predisposition to pancreatic cancer contributes significantly to its incidence and presents an opportunity for the development of early detection strategies. The genetic basis of predisposition remains unexplained in a high proportion of patients with familial PC (FPC). METHODS: Clinicopathologic features were assessed in a cohort of 766 patients who had been diagnosed with pancreatic ductal adenocarcinoma (PC). Patients were classified with FPC if they had ≥1 affected first-degree relatives; otherwise, they were classified with sporadic PC (SPC). RESULTS: The prevalence of FPC in this cohort was 8.9%. In FPC families with an affected parent-child pair, 71% in the subsequent generation were 12.3 years younger at diagnosis. Patients with FPC had more first-degree relatives who had an extrapancreatic malignancy (EPM) (42.6% vs 21.2; P<.0001), particularly melanoma and endometrial cancer, but not a personal history of EPM. Patients with SPC were more likely to be active smokers, have higher cumulative tobacco exposure, and have fewer multifocal precursor lesions, but these were not associated with differences in survival. Long-standing diabetes mellitus (>2 years) was associated with poor survival in both groups. CONCLUSIONS: FPC represents 9% of PC, and the risk of malignancy in kindred does not appear to be confined to the pancreas. Patients with FPC have more precursor lesions and include fewer active smokers, but other clinicopathologic factors and outcome are similar to those in patients with SPC. Furthermore, some FPC kindreds may exhibit anticipation. A better understanding of the clinical features of PC will facilitate efforts to uncover novel susceptibility genes and the development of early detection strategies.
Keyword Pancreatic cancer
Hereditary
Epidemiology
Prognosis
Q-Index Code C1
Q-Index Status Confirmed Code
Institutional Status UQ

Document type: Journal Article
Sub-type: Article (original research)
Collections: Official 2015 Collection
Institute for Molecular Bioscience - Publications
 
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Created: Thu, 27 Nov 2014, 21:11:42 EST by Katrina Garner-Moore on behalf of Institute for Molecular Bioscience