학술논문
Pancreatic Cancer Surveillance in Carriers of a Germline CDKN2A Pathogenic Variant: Yield and Outcomes of a 20-Year Prospective Follow-Up.
Document Type
Academic Journal
Author
Klatte DCF; Department of Gastroenterology and Hepatology, Leiden University Medical Center, Leiden, the Netherlands.; Boekestijn B; Department of Radiology, Leiden University Medical Center, Leiden, the Netherlands.; Wasser MNJM; Department of Radiology, Leiden University Medical Center, Leiden, the Netherlands.; Feshtali Shahbazi S; Department of Radiology, Leiden University Medical Center, Leiden, the Netherlands.; Ibrahim IS; Department of Gastroenterology and Hepatology, Leiden University Medical Center, Leiden, the Netherlands.; Mieog JSD; Department of Surgery, Leiden University Medical Center, Leiden, the Netherlands.; Luelmo SAC; Department of Oncology, Leiden University Medical Center, Leiden, the Netherlands.; Morreau H; Department of Pathology, Leiden University Medical Center, Leiden, the Netherlands.; Potjer TP; Department of Clinical Genetics, Leiden University Medical Center, Leiden, the Netherlands.; Inderson A; Department of Gastroenterology and Hepatology, Leiden University Medical Center, Leiden, the Netherlands.; Boonstra JJ; Department of Gastroenterology and Hepatology, Leiden University Medical Center, Leiden, the Netherlands.; Dekker FW; Department of Clinical Epidemiology, Leiden University Medical Center, Leiden, the Netherlands.; Vasen HFA; Department of Gastroenterology and Hepatology, Leiden University Medical Center, Leiden, the Netherlands.; van Hooft JE; Department of Gastroenterology and Hepatology, Leiden University Medical Center, Leiden, the Netherlands.; Bonsing BA; Department of Radiology, Leiden University Medical Center, Leiden, the Netherlands.; van Leerdam ME; Department of Gastroenterology and Hepatology, Leiden University Medical Center, Leiden, the Netherlands.; Department of Gastrointestinal Oncology, Netherlands Cancer Institute, Amsterdam, the Netherlands.
Source
Publisher: American Society of Clinical Oncology Country of Publication: United States NLM ID: 8309333 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1527-7755 (Electronic) Linking ISSN: 0732183X NLM ISO Abbreviation: J Clin Oncol Subsets: MEDLINE
Subject
Language
English
Abstract
Purpose: Pancreatic cancer surveillance in high-risk individuals may lead to detection of pancreatic ductal adenocarcinoma (PDAC) at an earlier stage and with improved survival. This study evaluated the yield and outcomes of 20 years of prospective surveillance in a large cohort of individuals with germline pathogenic variants (PVs) in CDKN2A .
Methods: Prospectively collected data were analyzed from individuals participating in pancreatic cancer surveillance. Surveillance consisted of annual magnetic resonance imaging with magnetic resonance cholangiopancreatography and optional endoscopic ultrasound.
Results: Three hundred forty-seven germline PV carriers participated in surveillance and were followed for a median of 5.6 (interquartile range 2.3-9.9) years. A total of 36 cases of PDAC were diagnosed in 31 (8.9%) patients at a median age of 60.4 (interquartile range 51.3-64.1) years. The cumulative incidence of primary PDAC was 20.7% by age 70 years. Five carriers (5 of 31; 16.1%) were diagnosed with a second primary PDAC. Thirty (83.3%) of 36 PDACs were considered resectable at the time of imaging. Twelve cases (12 of 36; 33.3%) presented with stage I disease. The median survival after diagnosis of primary PDAC was 26.8 months, and the 5-year survival rate was 32.4% (95% CI, 19.1 to 54.8). Individuals with primary PDAC who underwent resection (22 of 31; 71.0%) had an overall 5-year survival rate of 44.1% (95% CI, 27.2 to 71.3). Nine (2.6%; 9 of 347) individuals underwent surgery for a suspected malignant lesion, which proved to not be PDAC, and this included five lesions with low-grade dysplasia.
Conclusion: This long-term surveillance study demonstrates a high incidence of PDAC in carriers of a PV in CDKN2A . This provides evidence that surveillance in such a high-risk population leads to detection of early-stage PDAC with improved resectability and survival.
Methods: Prospectively collected data were analyzed from individuals participating in pancreatic cancer surveillance. Surveillance consisted of annual magnetic resonance imaging with magnetic resonance cholangiopancreatography and optional endoscopic ultrasound.
Results: Three hundred forty-seven germline PV carriers participated in surveillance and were followed for a median of 5.6 (interquartile range 2.3-9.9) years. A total of 36 cases of PDAC were diagnosed in 31 (8.9%) patients at a median age of 60.4 (interquartile range 51.3-64.1) years. The cumulative incidence of primary PDAC was 20.7% by age 70 years. Five carriers (5 of 31; 16.1%) were diagnosed with a second primary PDAC. Thirty (83.3%) of 36 PDACs were considered resectable at the time of imaging. Twelve cases (12 of 36; 33.3%) presented with stage I disease. The median survival after diagnosis of primary PDAC was 26.8 months, and the 5-year survival rate was 32.4% (95% CI, 19.1 to 54.8). Individuals with primary PDAC who underwent resection (22 of 31; 71.0%) had an overall 5-year survival rate of 44.1% (95% CI, 27.2 to 71.3). Nine (2.6%; 9 of 347) individuals underwent surgery for a suspected malignant lesion, which proved to not be PDAC, and this included five lesions with low-grade dysplasia.
Conclusion: This long-term surveillance study demonstrates a high incidence of PDAC in carriers of a PV in CDKN2A . This provides evidence that surveillance in such a high-risk population leads to detection of early-stage PDAC with improved resectability and survival.