Gastroenteropancreatic neuroendocrine neoplasms: A clinical snapshot
Autor: | Nisha Nigil Haroon, Biju Pottakkat, Annu Susan George, Cornelius James Fernandez, Joseph M Pappachan, Mayuri Agarwal |
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Rok vydání: | 2021 |
Předmět: |
Oncology
medicine.medical_specialty Vasoactive intestinal peptide Gastroenteropancreatic neuroendocrine neoplasms Enteroendocrine cell Review Targeted molecular therapy Metastasis 03 medical and health sciences 0302 clinical medicine Neuroendocrine tumours Targeted Molecular Therapy Internal medicine medicine Chemotherapy Pancreatic polypeptide business.industry medicine.disease Surgery Somatostatin Octreoscan Calcitonin 030220 oncology & carcinogenesis Neuroendocrine carcinoma 030211 gastroenterology & hepatology Differential diagnosis business hormones hormone substitutes and hormone antagonists |
Zdroj: | World Journal of Gastrointestinal Surgery |
ISSN: | 1948-9366 |
DOI: | 10.4240/wjgs.v13.i3.231 |
Popis: | Our understanding about the epidemiological aspects, pathogenesis, molecular diagnosis, and targeted therapies of neuroendocrine neoplasms (NENs) have drastically advanced in the past decade. Gastroenteropancreatic (GEP) NENs originate from the enteroendocrine cells of the embryonic gut which share common endocrine and neural differentiation factors. Most NENs are well-differentiated, and slow growing. Specific neuroendocrine biomarkers that are used in the diagnosis of functional NENs include insulin, glucagon, vasoactive intestinal polypeptide, gastrin, somatostatin, adrenocorticotropin, growth hormone releasing hormone, parathyroid hormone-related peptide, serotonin, histamine, and 5-hydroxy indole acetic acid (5-HIAA). Biomarkers such as pancreatic polypeptide, human chorionic gonadotrophin subunits, neurotensin, ghrelin, and calcitonin are used in the diagnosis of non-functional NENs. 5-HIAA levels correlate with tumour burden, prognosis and development of carcinoid heart disease and mesenteric fibrosis, however several diseases, medications and edible products can falsely elevate the 5-HIAA levels. Organ-specific transcription factors are useful in the differential diagnosis of metastasis from an unknown primary of well-differentiated NENs. Emerging novel biomarkers include circulating tumour cells, circulating tumour DNA, circulating micro-RNAs, and neuroendocrine neoplasms test (NETest) (simultaneous measurement of 51 neuroendocrine-specific marker genes in the peripheral blood). NETest has high sensitivity (85%-98%) and specificity (93%-97%) for the detection of gastrointestinal NENs, and is useful for monitoring treatment response, recurrence, and prognosis. In terms of management, surgery, radiofrequency ablation, symptom control with medications, chemotherapy and molecular targeted therapies are all considered as options. Surgery is the mainstay of treatment, but depends on factors including age of the individual, location, stage, grade, functional status, and the heredity of the tumour (sporadic vs inherited). Medical management is helpful to alleviate the symptoms, manage inoperable lesions, suppress postoperative tumour growth, and manage recurrences. Several molecular-targeted therapies are considered second line to somatostatin analogues. This review is a clinical update on the pathophysiological aspects, diagnostic algorithm, and management of GEP NENs. |
Databáze: | OpenAIRE |
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