title: Prevalence of familial pancreatic cancer in Germany.
abstract: Based on several case-control studies, it has been estimated that familial aggregation and genetic susceptibility play a role in up to 10% of patients with pancreatic cancer, although conclusive epidemiologic data are still lacking. Therefore, we evaluated the prevalence of familial pancreatic cancer and differences to its sporadic form in a prospective multicenter trial. A total of 479 consecutive patients with newly diagnosed, histologically confirmed adenocarcinoma of the pancreas were prospectively evaluated regarding medical and family history, treatment and pathology of the tumour. A family history for pancreatic cancer was confirmed whenever possible by reviewing the tumour specimens and medical reports. Statistical analysis was performed by calculating odds ratios, regression analysis with a logit-model and the Kaplan-Meier method. Twenty-three of 479 (prevalence 4.8%, 95% CI 3.1-7.1) patients reported at least 1 first-degree relative with pancreatic cancer. The familial aggregation could be confirmed by histology in 5 of 23 patients (1.1%, 95% CI 0.3-2.4), by medical records in 9 of 23 patients (1.9%, 95% CI 0.9-3.5) and by standardized interviews of first-degree relatives in 17 of 23 patients (3.5%, 95% CI 2.1-5.6), respectively. There were no statistical significant differences between familial and sporadic pancreatic cancer cases regarding sex ratio, age of onset, presence of diabetes mellitus and pancreatitis, tumour histology and stage, prognosis after palliative or curative treatment as well as associated tumours in index patients and families, respectively. The prevalence of familial pancreatic cancer in Germany is at most 3.5% (range 1.1-3.5%) depending on the mode of confirmation of the pancreatic carcinoma in relatives. This prevalence is lower than so far postulated in the literature. There were no significant clinical differences between the familial and sporadic form of pancreatic cancer.
authors.affiliation: National Case Collection of Familial Pancreatic Cancer, Department of Surgery, Philipps-University Marburg, Baldingerstrasse, 35043 Marburg, Germany. bartsch@mailer.uni-marburg.de
authors.forename: Detlef K
authors.initials: DK
authors.lastname: Bartsch
authors.forename: Ralf
authors.initials: R
authors.lastname: Kress
authors.forename: Mercedes
authors.initials: M
authors.lastname: Sina-Frey
authors.forename: Robert
authors.initials: R
authors.lastname: Grützmann
authors.forename: Berthold
authors.initials: B
authors.lastname: Gerdes
authors.forename: Christian
authors.initials: C
authors.lastname: Pilarsky
authors.forename: Joachim W
authors.initials: JW
authors.lastname: Heise
authors.forename: Klaus-Martin
authors.initials: KM
authors.lastname: Schulte
authors.forename: Mario
authors.initials: M
authors.lastname: Colombo-Benkmann
authors.forename: Cristina
authors.initials: C
authors.lastname: Schleicher
authors.forename: Helmut
authors.initials: H
authors.lastname: Witzigmann
authors.forename: Olaf
authors.initials: O
authors.lastname: Pridöhl
authors.forename: Michael B
authors.initials: MB
authors.lastname: Ghadimi
authors.forename: Olaf
authors.initials: O
authors.lastname: Horstmann
authors.forename: Wolfgang
authors.initials: W
authors.lastname: von Bernstorff
authors.forename: Lisa
authors.initials: L
authors.lastname: Jochimsen
authors.forename: Jan
authors.initials: J
authors.lastname: Schmidt
authors.forename: Sven
authors.initials: S
authors.lastname: Eisold
authors.forename: Lope
authors.initials: L
authors.lastname: Estévéz-Schwarz
authors.forename: Stephan A
authors.initials: SA
authors.lastname: Hahn
authors.forename: Karsten
authors.initials: K
authors.lastname: Schulmann
authors.forename: Wolfgang
authors.initials: W
authors.lastname: Böck
authors.forename: Thomas M
authors.initials: TM
authors.lastname: Gress
authors.forename: Nikolaus
authors.initials: N
authors.lastname: Zügel
authors.forename: Karl
authors.initials: K
authors.lastname: Breitschaft
authors.forename: Klaus
authors.initials: K
authors.lastname: Prenzel
authors.forename: Helmut
authors.initials: H
authors.lastname: Messmann
authors.forename: Esther
authors.initials: E
authors.lastname: Endlicher
authors.forename: Margarete
authors.initials: M
authors.lastname: Schneider
authors.forename: Andreas
authors.initials: A
authors.lastname: Ziegler
authors.forename: Wolff
authors.initials: W
authors.lastname: Schmiegel
authors.forename: Helmut
authors.initials: H
authors.lastname: Schäfer
authors.forename: Matthias
authors.initials: M
authors.lastname: Rothmund
authors.forename: Harald
authors.initials: H
authors.lastname: Rieder
mesh_terms: D000328:Adult; D000368:Aged; D005190:Family; D005260:Female; D020022:Genetic Predisposition to Disease / Q000235:genetics*; D005858:Germany / Q000453:epidemiology; D006801:Humans; D008297:Male; D008499:Medical Records; D008875:Middle Aged; D016017:Odds Ratio; D010190:Pancreatic Neoplasms / Q000453:epidemiology* / Q000235:genetics* / Q000473:pathology; D015995:Prevalence; D012189:Retrospective Studies