PMID- 19627764 OWN - NLM STAT- MEDLINE DCOM- 20110201 LR - 20090724 IS - 1579-2021 (Electronic) IS - 1575-0922 (Linking) VI - 56 Suppl 2 DP - 2009 Apr TI - [Associated gastroenteropancreatic neuroendocrine tumours to familiar syndromes]. PG - 10-5 LID - 10.1016/S1575-0922(09)70859-9 [doi] AB - Aproximately 5-10% of neuroendocrine tumours (NETs) of the gastroenteropancreatic system (GEP) have an hereditary background. The known hereditary syndromes include: multiple endocrine neoplasia type 1 (MEN 1), von Hippel Lindau disease (VHL), neurofibromatosis type 1 (NF 1) and tuberous sclerosis complex (TSC). This review discusses for each of these syndromes the: genes involved and specifics types of mutations, disease prevalence, affected neuroendocrine tissues and related clinical syndromes, and special morphological features of NETs on each of these syndromes. FAU - Lecumberri Pascual, Edurne AU - Lecumberri Pascual E AD - Servicio de Endocrinologia y Nutricion, Fundacion Jimenez Diaz Capio, Madrid, Espana. edurnelecumberri@hotmail.com LA - spa PT - English Abstract PT - Journal Article PT - Review TT - Tumores neuroendocrinos gastroenteropancreaticos asociados a sindromes familiares. DEP - 20090518 PL - Spain TA - Endocrinol Nutr JT - Endocrinologia y nutricion : organo de la Sociedad Espanola de Endocrinologia y Nutricion JID - 100886482 RN - Gastro-enteropancreatic neuroendocrine tumor SB - IM MH - Humans MH - Intestinal Neoplasms/genetics MH - Multiple Endocrine Neoplasia Type 1/genetics MH - Neoplasms, Multiple Primary/*genetics MH - Neoplastic Syndromes, Hereditary/*genetics MH - Neuroendocrine Tumors/genetics MH - Pancreatic Neoplasms/genetics MH - Stomach Neoplasms/genetics EDAT- 2009/07/25 09:00 MHDA- 2011/02/02 06:00 CRDT- 2009/07/25 09:00 PHST- 2009/07/25 09:00 [entrez] PHST- 2009/07/25 09:00 [pubmed] PHST- 2011/02/02 06:00 [medline] AID - S1575-0922(09)70859-9 [pii] AID - 10.1016/S1575-0922(09)70859-9 [doi] PST - ppublish SO - Endocrinol Nutr. 2009 Apr;56 Suppl 2:10-5. doi: 10.1016/S1575-0922(09)70859-9. Epub 2009 May 18.