PMID- 16183525 OWN - NLM STAT- MEDLINE DCOM- 20060117 LR - 20220310 IS - 1521-6918 (Print) IS - 1521-6918 (Linking) VI - 19 IP - 4 DP - 2005 Aug TI - Endocrine tumours of the gastrointestinal tract: aetiology, molecular pathogenesis and genetics. PG - 519-34 AB - Endocrine tumours of the gut and pancreas originate from cells of the diffuse endocrine system and are characterised by the production of a wide variety of bioactive substances including growth factors. Two major tumour categories are distinguished-well-differentiated and poorly differentiated neoplasms-with distinct phenotypes and significantly diverse clinical behaviour. Here, genetic background data are summarised on an anatomical basis for tumours of foregut, midgut and hindgut derivatives. For well-differentiated tumours, independent techniques identified the abnormality of multiple chromosomal sites and genes, pointing to a complex genetic background. Differences in foregut tumours compared with midgut and hindgut tumours are, however, outlined. The multiple endocrine neoplasia syndrome type 1 (MEN1) gene is reported to be involved in about one-third of sporadic foregut endocrine tumours and exceptionally in midgut and hindgut tumours. Similarly, X chromosome markers are associated with malignant behaviour in foregut tumours only. For poorly differentiated carcinomas, a high degree of chromosomal instability is the common genetic trait independent of tumour site and frequently involving the p53 gene. FAU - Rindi, Guido AU - Rindi G AD - Department of Pathology and Laboratory Medicine, Section of Anatomic Pathology, University of Parma, Italy. guido.rindi@unipr.it FAU - Bordi, Cesare AU - Bordi C LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review PL - Netherlands TA - Best Pract Res Clin Gastroenterol JT - Best practice & research. Clinical gastroenterology JID - 101120605 RN - 0 (MEN1 protein, human) RN - 0 (Proto-Oncogene Proteins) RN - 0 (RASSF1 protein, human) RN - 0 (Tumor Suppressor Proteins) RN - 76057-06-2 (Transforming Growth Factors) SB - IM MH - Carcinoma, Neuroendocrine/*genetics/pathology MH - Cell Differentiation MH - Gastrointestinal Neoplasms/*genetics/pathology MH - Genes, Tumor Suppressor/physiology MH - Humans MH - Immunohistochemistry MH - Loss of Heterozygosity MH - Methylation MH - Pancreatic Neoplasms/*genetics/pathology MH - Phenotype MH - Proto-Oncogene Proteins/genetics/physiology MH - Transforming Growth Factors/physiology MH - Tumor Suppressor Proteins/genetics RF - 105 EDAT- 2005/09/27 09:00 MHDA- 2006/01/18 09:00 CRDT- 2005/09/27 09:00 PHST- 2005/09/27 09:00 [pubmed] PHST- 2006/01/18 09:00 [medline] PHST- 2005/09/27 09:00 [entrez] AID - S1521-6918(05)00064-8 [pii] AID - 10.1016/j.bpg.2005.03.005 [doi] PST - ppublish SO - Best Pract Res Clin Gastroenterol. 2005 Aug;19(4):519-34. doi: 10.1016/j.bpg.2005.03.005.