PMID- 15606375 OWN - NLM STAT- MEDLINE DCOM- 20050214 LR - 20061115 IS - 0954-6820 (Print) IS - 0954-6820 (Linking) VI - 257 IP - 1 DP - 2005 Jan TI - Surgery for gastroenteropancreatic tumours in multiple endocrine neoplasia type 1: review and personal experience. PG - 38-49 AB - Multiple endocrine neoplasia type 1 (MEN1) gastro-entero-pancreatic (GEP) tumours develop from the pancreatic islets and from the endocrine cells of the duodenal and gastric mucosa. Even if GEP tumours have generally a benign course, a subgroup of them shows an aggressive behaviour and is a major cause of death amongst MEN1 patients. Diagnosis of insulinoma should lead promptly to pancreatic surgery. MEN1 gastrinomas are multiple and almost exclusively localized in the duodenum. Cure rate for Zollinger-Ellison syndrome in MEN1 is low when surgery is limited to tumour enucleation or full thickness duodenal wall resection. Conversely, pancreatoduodenectomy is followed by higher chance of cure. For nonfunctioning tumours exceeding 1 cm diameter in size a prompt treatment is recommended due to their high malignant potential. Gastroscopic surveillance is indicated for the frequent occurrence of multiple, small, type 2 fundic carcinoids. Endoscopic removal is possible for lesions growing in the mucosa-submucosa, but partial or even total gastrectomy is recommended for the small number of gastric carcinoids infiltrating the muscular layers. FAU - Tonelli, F AU - Tonelli F AD - Department of Clinical Physiopathology, University of Florence, Florence, Italy. f.tonelli@dfc.unifi.it FAU - Fratini, G AU - Fratini G FAU - Falchetti, A AU - Falchetti A FAU - Nesi, G AU - Nesi G FAU - Brandi, M L AU - Brandi ML LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review PL - England TA - J Intern Med JT - Journal of internal medicine JID - 8904841 SB - IM MH - Gastrinoma/surgery MH - Gastrointestinal Neoplasms/*surgery MH - Humans MH - Insulinoma/surgery MH - Multiple Endocrine Neoplasia Type 1/*surgery MH - Neoplasm Metastasis/therapy MH - Pancreatic Neoplasms/surgery MH - Pancreaticoduodenectomy/methods MH - Prognosis MH - Stomach Neoplasms/surgery MH - Time Factors RF - 75 EDAT- 2004/12/21 09:00 MHDA- 2005/02/16 09:00 CRDT- 2004/12/21 09:00 PHST- 2004/12/21 09:00 [pubmed] PHST- 2005/02/16 09:00 [medline] PHST- 2004/12/21 09:00 [entrez] AID - JIM1424 [pii] AID - 10.1111/j.1365-2796.2004.01424.x [doi] PST - ppublish SO - J Intern Med. 2005 Jan;257(1):38-49. doi: 10.1111/j.1365-2796.2004.01424.x.