Recent advances in diagnosis and treatment of gastroenteropancreatic neuroendocrine neoplasms.
Autor: | Dai M; Clinic of General Surgery, Molecular Oncology and Immunotherapy, Rostock University Medical Center, 18057 Rostock, Germany., Mullins CS; Clinic of General Surgery, Molecular Oncology and Immunotherapy, Rostock University Medical Center, 18057 Rostock, Germany., Lu L; Clinic of General Surgery, Molecular Oncology and Immunotherapy, Rostock University Medical Center, 18057 Rostock, Germany., Alsfasser G; Clinic of General Surgery, Rostock University Medical Center, 18057 Rostock, Germany., Linnebacher M; Clinic of General Surgery, Molecular Oncology and Immunotherapy, Rostock University Medical Center, 18057 Rostock, Germany. michael.linnebacher@med.uni-rostock.de. |
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Jazyk: | angličtina |
Zdroj: | World journal of gastrointestinal surgery [World J Gastrointest Surg] 2022 May 27; Vol. 14 (5), pp. 383-396. |
DOI: | 10.4240/wjgs.v14.i5.383 |
Abstrakt: | Gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs) are a rare group of tumors originating from neuroendocrine cells of the digestive system. Their incidence has increased over the last decades. The specific pathogenetic mechanisms underlying GEP-NEN development have not been completely revealed. Unfunctional GEP-NENs are usually asymptomatic; some grow slowly and thus impede early diagnosis, which ultimately results in a high rate of misdiagnosis. Therefore, many GEP-NEN patients present with later staged tumors. Motivated hereby, research attention for diagnosis and treatment for GEP-NENs increased in recent years. The result of which is great progress in clinical diagnosis and treatment. According to the most recent clinical guidelines, improved grading standards can accurately define poorly differentiated grade 3 neuroendocrine tumors and neuroendocrine carcinomas (NECs), which are subclassified into large and small cell NECs. Combining different functional imaging methods facilitates precise diagnosis. The expression of somatostatin receptors helps to predict prognosis. Genetic analyses of mutations affecting death domain associated protein ( DAXX ), multiple endocrine neoplasia type 1 ( MEN 1 ), alpha thalassemia/intellectual disability syndrome X-linked ( ATRX ), retinoblastoma transcriptional corepressor 1 ( RB 1 ), and mothers against decapentaplegic homolog 4 ( SMAD 4 ) help distinguishing grade 3 NENs from poorly differentiated NECs. The aim of this review is to summarize the latest research progress on diagnosis and treatment of GEP-NENs. Competing Interests: Conflict-of-interest statement: None to be declared. (©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.) |
Databáze: | MEDLINE |
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