Acinar cell carcinoma of the pancreas: a clinicopathologic and cytomorphologic review

Acinar cell carcinoma (ACC) is a rare malignancy of the pancreas with unique clinical, molecular, and morphologic characteristics. Clinically, these cancers can present with hypersecretory syndrome caused by the release of lipase into the circulation. Surgical resection is the treatment of choice for patients with organ-confined disease; however, with recent advances in precision medicine, therapies targeting the distinct molecular profile of ACC are on the horizon. Cytomorphologic features of ACC have been well described in the literature; and in conjunction with available clinical data and appropriate ancillary studies, an accurate diagnosis can be rendered in most instances. The aim of the current article is to present a comprehensive review of ACC based on available literature while attempting to shed light on some of its key histologic and cytologic characteristics.