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J Gastrointest Surg. 2019 Apr;23(4):768-778. doi: 10.1007/s11605-019-04113-3. Epub 2019 Jan 31.

Natural History and Treatment Trends in Pancreatic Cancer Subtypes.

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Department of Surgery, Columbia University Medical Center, New York, NY, USA.
Department of Surgery, University of Wisconsin, 600 Highland Avenue, H4/710 Clinical Science Center, Madison, WI, 53792-3284, USA.
Department of Pathology, University of Wisconsin, Madison, WI, USA.
Department of Surgery, University of Wisconsin, 600 Highland Avenue, H4/710 Clinical Science Center, Madison, WI, 53792-3284, USA.



While pancreatic ductal adenocarcinoma is the most common form of pancreatic cancer, many other histologic forms of pancreatic cancer are also recognized. These histologic variants portray unique characteristics in terms of patient demographics, tumor behavior, survival, and responsiveness to treatments.


Patients who underwent surgical resection of the pancreas for non-metastatic, invasive pancreatic cancer between 2004 and 2014 were selected from the National Cancer Data Base and categorized by histologic variant according to WHO classification guidelines. Patient demographics, tumor variables, treatment characteristics, and survival were compared between histologic groups and subgroups.


A total of 57,804 patients met inclusion and exclusion criteria and were grouped into eight major histologic categories. Survival analysis by the histologic group showed median overall survival of 20.2 months for ductal adenocarcinoma, 20.5 months for squamous cell carcinoma, 26.8 months for mixed acinar-neuroendocrine carcinomas, 52.6 months for cystic mucinous neoplasms with an associated invasive carcinoma, 67.5 months for acinar cell carcinoma, and 69.3 months for mesenchymal tumors. Median survival was not reached for neuroendocrine tumors and solid-pseudopapillary neoplasms, with 5-year overall survival rates of 84% and 97% respectively.


Rare subtypes of pancreatic cancer present unique clinicopathologic characteristics and display distinct tumor biologies. This study presents data on demographic, prognostic, treatment, and survival outcomes between rare forms of pancreatic neoplasms in order to aid understanding of the natural history and behavior of these neoplasms, with the hope of serving as a reference in clinical decision-making and ability to provide accurate prognostic information to patients.


Mucinous neoplasms; Neuroendocrine tumors; Pancreatic adenocarcinoma; Pancreatic cancer subtypes


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