Pancreatic Ductal Adenocarcinoma and Its Variants: Pearls and Perils

Pancreatic ductal adenocarcinoma (PDAC), an epithelial neoplasm derived from the pancreatic ductal tree, is the most common histologic type of pancreatic cancer and accounts for 85%-95% of all solid pancreatic tumors. As a highly lethal malignancy, it is the seventh leading cause of cancer death worldwide and is responsible for more than 300 000 deaths per year. PDAC is highly resistant to current therapies, affording patients a 5-year overall survival rate of only 7.2%. It is characterized histologically by its highly desmoplastic stroma embedding tubular and ductlike structures. On images, it typically manifests as a poorly defined hypoenhancing mass, causing ductal obstruction and vascular involvement. Little is known about the other histologic subtypes of PDAC, mainly because of their rarity and lack of specific patterns of disease manifestation. According to the World Health Organization, these variants include adenosquamous carcinoma, colloid carcinoma, hepatoid carcinoma, medullary carcinoma, signet ring cell carcinoma, undifferentiated carcinoma with osteoclast-like giant cells, and undifferentiated carcinoma. Depending on the subtype, they can confer a better or even worse prognosis than that of conventional PDAC. Thus, awareness of the existence and differentiation of these variants on the basis of imaging and histopathologic characteristics is crucial to guide clinical decision making for optimal treatment and patient management.