Pathology of gastrointestinal disorders

Nonneoplastic and neoplastic proliferative lesions of endocrine cells of the gastrointestinal tract are detailed. A multistep continuum from hyperplasia, dysplasia to neoplasia is identified for histamine-producing enterochromaffin-like (ECL) cells of the gastric corpus. Most gastric neuroendocrine tumors (NETs) are silent and composed by ECL cells, the second most frequent neuroendocrine neoplasms being the high-grade neuroendocrine carcinoma (NEC). In the duodenum, preneoplastic lesions are similarly described for gastrin (G) and somatostatin (D) cells. G-cell NETs are the most frequent neuroendocrine tumors of the duodenum, either functioning or nonfunctioning, followed by D-cell NETs and gangliocytic paraganglioma (GCP). No systematic definition of nonneoplastic lesions exists for endocrine cells of the ileum, appendix, and colon-rectum. The most frequent ileal NETs are serotonin-producing enterochromaffin (EC)-cell NETs (classic carcinoid), associating with functional syndrome only in presence of liver metastases. Neoplasms are usually larger in the colon as compared with the small lesions observed in the rectum. High-grade NECs are observed in the colon and rectum-sigmoid, often associate with nonendocrine neoplastic components, and fare an aggressive course with poor outcome and short survival.