Familial pancreatic cancer syndromes

被引：36

作者：

Habbe, Nils

Langer, Peter

Sina-Frey, Mercedes

Bartsch, Detlef K.

机构：

[1] Stadt Kliniken Bielefeld Mitte, Dept Surg, D-33042 Bielefeld, Germany

[2] Univ Marburg, Dept Surg, D-35033 Marburg, Germany

[3] Univ Marburg, Inst Clin Genet, D-35037 Marburg, Germany

来源：

ENDOCRINOLOGY AND METABOLISM CLINICS OF NORTH AMERICA | 2006年 / 35卷 / 02期

关键词：

D O I：

10.1016/j.ecl.2006.02.016

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Hereditary pancreatic cancer (PC) is rare and extremely heterogeneous, and it accounts for approximately 2% of all PC cases. The major component of hereditary PC is the familial pancreatic cancer syndrome. Although up to 20% of hereditary PC cases are associated with germline mutations in the BRCA2, CDKN2A, PRSS1, STK11 or MMR genes, the major underlying gene defect(s) is still unknown. Although hereditary PC is rare, the data on PC families that have been collected by various study groups worldwide provide a unique opportunity to evaluate the natural history, causative gene alterations, new diagnosis and chemoprevention strategies as well as treatment modalities.

引用

页码：417 / +

页数：15

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