UpToDate
Official reprint from UpToDate®
www.uptodate.com ©2017 UpToDate, Inc. and/or its affiliates. All Rights Reserved.

Medline ® Abstract for Reference 81

of 'Familial risk factors for pancreatic cancer and screening of high-risk patients'

81
TI
Benefit of Surveillance for Pancreatic Cancer in High-Risk Individuals: Outcome of Long-Term Prospective Follow-Up Studies From Three European Expert Centers.
AU
Vasen H, Ibrahim I, Ponce CG, Slater EP, Matthäi E, Carrato A, Earl J, Robbers K, van Mil AM, Potjer T, Bonsing BA, de Vos Tot Nederveen Cappel WH, Bergman W, Wasser M, Morreau H, Klöppel G, Schicker C, Steinkamp M, Figiel J, Esposito I, Mocci E, Vazquez-Sequeiros E, Sanjuanbenito A, Muñoz-Beltran M, Montans J, Langer P, Fendrich V, Bartsch DK
SO
J Clin Oncol. 2016;34(17):2010.
 
PURPOSE: 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. Surveillanceof 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 METHODS: 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.
RESULTS: 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.
CONCLUSION: 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.
AD
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 JoséMontans, 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.
PMID