Abstract

Primary squamous cell carcinoma is a particularly rare form of pancreatic cancer, comprising approximately 1% of all pancreatic malignancies, with a reported incidence range of 0.5% to 5%. This presents a clinical challenge as diagnosis, response to treatment, prognosis and outcome are relatively poorly understood compared the relatively more common pancreatic adenocarcinoma. Primary squamous cell carcinoma of the pancreas is typically only diagnosed once other primary sources of the tumor are excluded and histology confirms a lack of glandular component. Clinical information relating to primary squamous cell carcinoma is scant and is derived primarily from retrospective studies. Discrepancies in the reported incidence of primary squamous cell carcinoma in the literature have been attributed to erroneous categorization of adenosquamous carcinoma as primary squamous cell carcinoma, possibly leading to overestimation of incidence. Analyses of the biologic behavior of primary squamous cell carcinoma from previous reports highlights its propensity to affect older individuals, presence of metastasis at diagnosis, poor response to chemotherapeutic agents and radiation therapy and an extremely short survival period. Surgical resection is the only potential curative treatment and should be considered in all primary squamous cell carcinoma patients with resectable disease.