Diagnosis and Management of Gastrointestinal Neuroendocrine Tumors: A Comprehensive Literature Review

Abstract

Neuroendocrine tumors (NETs) are epithelial neoplasms with predominant neuroendocrine differentiation and the ability to synthesize and secrete variable hormones and monoamines. They are relatively rare, accounting for 2% of all malignancy cases in the United States. The most common system affected by NETs is the gastrointestinal tract. Clinical presentation depends on the organ being involved and the hormone being secreted. It can be variable from asymptomatic incidental findings on imaging to intestinal obstruction, or carcinoid syndrome (CS). Several biochemical testings are developed to help with the diagnosis of NETs including 5-hydroxyindoleacetic acid (5-HIAA) and chromogranin A (CgA). Computerized tomography (CT) scans and magnetic resonance imaging (MRI) are the most commonly used modalities to localize the primary tumor and evaluate for metastasis. However, radionuclide imaging using somatostatin receptor-based imaging techniques has improved accuracy to detect smaller neoplasm. Surgical removal is the mainstay of treatment for locoregional tumors. Several medical managements are available for non-respectable NETs which include SSAs, peptide receptor radionuclide therapy (PRRT), and platinum-based chemotherapy agents.

Keywords: 5-hiaa; carcinoid syndrome; chromgranin; functional neuroendocrine tumor; ga-68 dotatate scan; gastrointestinal neuroendocrine tumor; gi malignancy; peptide receptor radiation therapy(prrt).

Figure 1. Schematic mechanism of action for peptide receptor radionuclide therapy