• Hans Vasen, Isaura Ibrahim, Carmen Guillen Ponce, Emily P Slater, Elvira Matthäi, Alfredo Carrato, Julie Earl, Kristin Robbers, Anneke M van Mil, Thomas Potjer, Bert A Bonsing, Wouter H de Vos Tot Nederveen Cappel, Wilma Bergman, Martin Wasser, Hans Morreau, Günter Klöppel, Christoph Schicker, Martin Steinkamp, Jens Figiel, Irene Esposito, Evelina Mocci, Enrique Vazquez-Sequeiros, Alfonso Sanjuanbenito, Maria Muñoz-Beltran, José Montans, Peter Langer, Volker Fendrich, and Detlef K Bartsch.
• Hans Vasen, Isaura Ibrahim, Kristin Robbers, Anneke M. van Mil, Thomas Potjer, Bert A. Bonsing, Wilma Bergman, Martin Wasser, and Hans Morreau, Leiden University Medical Center, Leiden; Wouter H. de Vos tot Nederveen Cappel, Isala Clinics, Zwolle, the Netherlands; Carmen Guillen Ponce, Alfredo Carrato, Julie Earl, Evelina Mocci, Enrique Vazquez-Sequeiros, Alfonso Sanjuanbenito, Maria Muñoz-Beltran, and José Montans, Ramón y Cajal University Hospital, Ramón y Cajal Health Research Institute, Madrid, Spain; Emily P. Slater, Elvira Matthäi, Volker Fendrich, and Detlef K. Bartsch, University Hospital Marburg; Christoph Schicker, Martin Steinkamp, and Jens Figiel, Philipps University Marburg, Marburg; Günter Klöppel, Consultation Centre for Pancreatic and Endocrine Tumors, Technical University Munich; Peter Langer, Klinikum Hanau, Hanau, Germany; and Irene Esposito, Innsbruck University Hospital, Innsbruck, Austria. hfavasen@stoet.nl.
• J. Clin. Oncol. 2016 Jun 10; 34 (17): 2010-9.

PurposePancreatic 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.Patients And MethodsScreening 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.ResultsFour 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.ConclusionSurveillance 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.© 2016 by American Society of Clinical Oncology.

25 keywords...

hide…