ArticleDeutsches Arzteblatt international2021
The German National Case Collection for Familial Pancreatic Carcinoma (FaPaCa)—Knowledge Gained in 20 Years.
Article in Deutsches Arzteblatt international, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 15 papers.
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15 citing papers in PubMed, 20 citations in OpenAlex.
- Diagnosis and treatment strategies for hereditary pancreatic cancer syndrome.International journal of clinical oncology · 2025Review
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- Pancreatic cancer screening is effective in individuals at risk with predisposing germline gene variants, but not in gene variant-negative familial pancreatic cancer families.United European gastroenterology journal · 2024Article
- The Italian registry of families at risk for pancreatic cancer (IRFARPC): implementation and evolution of a national program for pancreatic cancer surveillance in high-risk individuals.Familial cancer · 2024Review
- Surgical aspects related to hereditary pancreatic cancer.Familial cancer · 2024Review
- The role of endoscopic ultrasound in the detection of pancreatic lesions in high-risk individuals.Familial cancer · 2024Review
- Aspects and outcomes of surveillance for individuals at high-risk of pancreatic cancer.Familial cancer · 2024Review
- Review
- Characteristics of familial pancreatic cancer families with additional colorectal carcinoma.Familial cancer · 2023Article
- [Familial pancreatic cancer syndrome].Chirurgie (Heidelberg, Germany) · 2023Review
- Circulating Cell-Free Nucleic Acids as Biomarkers for Diagnosis and Prognosis of Pancreatic Cancer.Biomedicines · 2023Review
- Proteomics biomarker discovery for individualized prevention of familial pancreatic cancer using statistical learning.PloS one · 2023Article
- Current status of inherited pancreatic cancer.Hereditary cancer in clinical practice · 2022Review
- Prevalence of Germline Pathogenic Variants in Cancer Predisposing Genes in Czech and Belgian Pancreatic Cancer Patients.Cancers · 2021Article
- Combinations of Low-Frequency Genetic Variants Might Predispose to Familial Pancreatic Cancer.Journal of personalized medicine · 2021Article
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Abstract
backgroundFamilial pancreatic carcinoma (FPC) is a rare hereditary tumor syndrome with a heterogeneous clinical phenotype. The study of FPC also contributes to a better understanding of the more common sporadic pancreatic ductal adenocarcinoma (PDAC). We report on the past 20 years' experience of the German National Case Collection for Familial Pancreatic Carcinoma (FaPaCa) of the German Cancer Aid (Deutsche Krebshilfe).
methodsFrom 1999 onward, families in which at least two first-degree relatives had PDAC, and which did not meet the criteria for any other tumor syndrome, have been entered into the FaPaCa registry and analyzed both clinically and with molecular genetic techniques. Persons at risk are offered the opportunity to participate in an early detection program.
resultsFrom June 1999 to June 2019, 227 families (a total of 2579 persons) met the criteria for entry into the FaPaCa registry. PDAC was the sole tumor entity present in 37% of the families (95% confidence interval [31.1; 44.1]); in the remaining 63% [55.9; 68.9], other tumor types were present as well, particularly breast cancer (70 families, 31% [24.9; 37.3]), colon carcinoma (25 families, 11% [7.3; 15.8]) , and melanoma (22 families, 9.7% [6.2; 14.3]). The mode of inheritance of PDAC was autosomal dominant in 72% [65.5; 77.6] of the families. Predisposing germ-line mutations were found in 25 of the 150 (16.7%) families studied, in the following genes: BRCA2 (9 families), CDKN2A (5 families), PALB2 (4 families), BRCA1 (3 families), ATM (2 families), and CHEK2 (2 families). The early detection program revealed high-grade cancer precursor lesions or a PDAC in 5 of the participating 110 persons at risk (4.5%, [1.5; 10.3] during a period of observation of at least five years.
conclusionThe care of families with FPC is complex and should be provided in centers with the necessary expertise. Prospective, controlled longitudinal studies are needed to determine whether the screening of persons at risk for PDAC truly lessens mortality and is cost-effective.
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