Pathology of Gastrointestinal Neuroendocrine Tumors: An Update

Gastrointestinal (GI) neuroendocrine tumors (NETs) are a heterogeneous group of relatively slow-growing neoplasms with marked site-specific differences in hormonal secretion and clinical behavior. Most are sporadic neoplasms, with only 5% to 10% arising in patients with hereditary disorders, most commonly in multiple endocrine neoplasia type 1. Although a uniform terminology is not universally accepted, use of the 4-category WHO classification of these tumors is becoming more widespread, and recommendations for tumor grading and staging have been recently formulated. Most GI NETs are easily recognized on routine histologic examination; rarely, a limited panel of immunohistochemical markers may be useful in establishing the diagnosis. This article describes general and site-specific features of these tumors and outlines potential pitfalls in diagnosis.