Diagnosis and Management of Gastrointestinal Neuroendocrine Neoplasms

Raul S Gonzalez

doi:10.1016/j.path.2020.04.002

Diagnosis and Management of Gastrointestinal Neuroendocrine Neoplasms

Surg Pathol Clin. 2020 Sep;13(3):377-397. doi: 10.1016/j.path.2020.04.002.

Author

Raul S Gonzalez¹

Affiliation

¹ Department of Pathology, Beth Israel Deaconess Medical Center, 330 Brookline Avenue, Boston, MA 02215, USA; Harvard Medical School, Boston, MA, USA. Electronic address: Rgonzal5@bidmc.harvard.edu.

PMID: 32773190
DOI: 10.1016/j.path.2020.04.002

Abstract

The latest WHO classification cleanly divides gastrointestinal neuroendocrine neoplasms into neuroendocrine tumor (NET; well-differentiated, any grade) and neuroendocrine carcinoma (NEC; poorly differentiated, high-grade by definition), along with mixed neuroendocrine-non-neuroendocrine neoplasms. NECs are always aggressive, with multiple mutations; they are treated with chemotherapy. NETs have widely different presentations, behavior, and management depending on site of origin. Esophageal examples are vanishingly rare. Most gastric and appendiceal tumors are indolent, as are many colonic and rectal tumors. The duodenum is home to some unusual variants of NET, and jejunal/ileal NETs frequently metastasize, which impacts their staging and clinical management.

Keywords: Gastrointestinal; Grading; Neuroendocrine carcinoma; Neuroendocrine neoplasms; Neuroendocrine tumor; Staging; WHO classification.

Publication types

Review

MeSH terms

Gastrointestinal Neoplasms / diagnosis*
Gastrointestinal Neoplasms / pathology
Gastrointestinal Neoplasms / therapy
Gastrointestinal Tract / pathology
Humans
Neoplasm Staging
Neuroendocrine Tumors / diagnosis*
Neuroendocrine Tumors / pathology
Neuroendocrine Tumors / therapy