Prevalence of familial pancreatic cancer in Germany.
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.
Bartsch
Detlef K
DK
National Case Collection of Familial Pancreatic Cancer, Department of Surgery, Philipps-University Marburg, Baldingerstrasse, 35043 Marburg, Germany. bartsch@mailer.uni-marburg.de
Kress
Ralf
R
Sina-Frey
Mercedes
M
Grützmann
Robert
R
Gerdes
Berthold
B
Pilarsky
Christian
C
Heise
Joachim W
JW
Schulte
Klaus-Martin
KM
Colombo-Benkmann
Mario
M
Schleicher
Cristina
C
Witzigmann
Helmut
H
Pridöhl
Olaf
O
Ghadimi
Michael B
MB
Horstmann
Olaf
O
von Bernstorff
Wolfgang
W
Jochimsen
Lisa
L
Schmidt
Jan
J
Eisold
Sven
S
Estévéz-Schwarz
Lope
L
Hahn
Stephan A
SA
Schulmann
Karsten
K
Böck
Wolfgang
W
Gress
Thomas M
TM
Zügel
Nikolaus
N
Breitschaft
Karl
K
Prenzel
Klaus
K
Messmann
Helmut
H
Endlicher
Esther
E
Schneider
Margarete
M
Ziegler
Andreas
A
Schmiegel
Wolff
W
Schäfer
Helmut
H
Rothmund
Matthias
M
Rieder
Harald
H
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