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Vasen, Hans; Ibrahim, Isaura; Ponce, Carmen Guillen; Slater, Emily P; Matthäi, Elvira; Carrato, Alfredo; Earl, Julie; Robbers, Kristin; van Mil, Anneke M; Potjer, Thomas; Bonsing, Bert A; de Vos Tot Nederveen Cappel, Wouter H; Bergman, Wilma; Wasser, Martin; Morreau, Hans; Klöppel, Günter; Schicker, Christoph; Steinkamp, Martin; Figiel, Jens; Esposito, Irene; Mocci, Evelina; Vazquez-Sequeiros, Enrique; Sanjuanbenito, Alfonso; Muñoz-Beltran, Maria; Montans, José; Langer, Peter; Fendrich, Volker; Bartsch, Detlef K
Journal of clinical oncology, 06/2016, Letnik: 34, Številka: 17Journal Article
Pancreatic ductal adenocarcinoma (PDAC) has a poor prognosis. Hereditary factors play a role in the development of PDAC in 3% to 5% of all patients. Surveillance of high-risk groups, may facilitate detection of PDAC at an early stage. The aim of this study was to assess whether surveillance aids detection of early-stage PDAC or precursor lesions (PRLs) and improves the prognosis. Screening outcomes were collected from three European centers that conduct prospective screening in high-risk groups including families with clustering of PDAC (familial pancreatic cancer FPC) or families with a gene defect that predisposes to PDAC. The surveillance program consisted of annual magnetic resonance imaging, magnetic resonance cholangiopancreatography, and/or endoscopic ultrasound. Four hundred eleven asymptomatic individuals participated in the surveillance programs, including 178 CDKN2A mutation carriers, 214 individuals with FPC, and 19 BRCA1/2 or PALB2 mutation carriers. PDAC was detected in 13 (7.3%) of 178 CDKN2A mutation carriers. The resection rate was 75%, and the 5-year survival rate was 24%. Two CDKN2A mutation carriers (1%) underwent surgical resection for low-risk PRL. Two individuals (0.9%) in the FPC cohort had a pancreatic tumor, including one advanced PDAC and one early grade 2 neuroendocrine tumor. Thirteen individuals with FPC (6.1%) underwent surgical resection for a suspected PRL, but only four (1.9%) had high-risk lesions (ie, high-grade intraductal papillary mucinous neoplasms or grade 3 pancreatic intraepithelial neoplasms). One BRCA2 mutation carrier was found to have PDAC, and another BRCA2 mutation carrier and a PALB2 mutation carrier underwent surgery and were found to have low-risk PRL. No serious complications occurred as consequence of the program. Surveillance of CDNK2A mutation carriers is relatively successful, detecting most PDACs at a resectable stage. The benefit of surveillance in families with FPC is less evident.
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JCR | SNIP | JCR | SNIP | JCR | SNIP | JCR | SNIP |
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