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• W2135370951 abstract "This classification can be used for all types of NE tumour; not only for carcinoids. A classical midgut carcinoid will, in the new terminology, be classified as a well-differentiated endocrine carcinoma of the ileum, whereas a benign insulin-producing tumour of the pancreas will be a well-differentiated endocrine tumour of the pancreas. The differentiation between different tumour types is based on histomorphology, tumour size and the presence or absence of local invasion and/or metastases. This new classification of NE tumours is a step forward, although the former classification of carcinoid tumours into foregut, midgut and hindgut remains clinically available and is still used in many clinical studies. It will take some time for the new classification to be generally accepted. NE tumours exhibit substantial differences in terms of genotype and phenotype. Foregut carcinoids mainly located in the lung but also endocrine pancreatic tumours, frequently show loss of 11q, which represent a characteristic genetic alteration in these tumours. Both typical and atypical carcinoids of the lung show loss of heterozygosity at 11q13, harbouring the multiple endocrine neoplasia type 1 (MEN-1) gene. Atypical carcinoids also show loss of heterozygosity at 3p14–p21.3. Recent studies have shown that carcinoid tumours of the lung and the gastrointestinal tract may develop via different molecular pathways. Inactivation of one of several tumour suppressor genes on chromosome 18 may be important for the biological behaviour of gastrointestinal tumours. Familial midgut carcinoids are rare but bronchial carcinoids as well as endocrine pancreatic tumours and gastric carcinoids may be part of a MEN-1 syndrome [4, 5]. Clinical presentation The different NE tumours may be divided into functioning and non-functioning tumours. Functioning tumours present clinically with symptoms related to overproduction of hormones and amines such as midgut carcinoids with carcinoid syndrome, gastrinoma with Zollinger–Ellison’s syndrome, insulinoma with hypoglycaemic symptoms, glucagonoma with glucagonoma syndrome and VIPoma with watery diarrhoea– hypokalaemia –achlorhydria (WDHA) syndrome. Non-functioning tumours produce and secrete peptides that do not cause any distinct clinical symptom. The majority of endocrine pancreatic tumours is non-functioning tumours (40–45%). The classical carcinoid syndrome includes flushing (80%), diarrhoea (70%), abdominal pain (40%), valvular heart disease (30–40%), telangiectasia (25%), wheezing (15%) and pellagralike skin lesions (5%)." @default.
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• W2135370951 date "2004-10-01" @default.
• W2135370951 modified "2023-10-12" @default.
• W2135370951 title "Management of neuroendocrine tumours" @default.
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• W2135370951 doi "https://doi.org/10.1093/annonc/mdh942" @default.
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