Hereditary neuroendocrine tumors of the gastroenteropancreatic system

被引：76

作者：

Anlauf, Martin

Garbrecht, Nele

Bauersfeld, Juliane

Schmitt, Anja

Henopp, Tobias

Komminoth, Paul

Heitz, Philipp U.

Perren, Aurel

Kloeppel, Guenter

机构：

[1] Univ Kiel, Dept Pathol, D-24105 Kiel, Germany

[2] Univ Zurich Hosp, Dept Pathol, CH-8091 Zurich, Switzerland

[3] Triemli Spital, Inst Pathol, Zurich, Switzerland

[4] Tech Univ Munich, Dept Pathol, D-8000 Munich, Germany

来源：

VIRCHOWS ARCHIV | 2007年 / 451卷

关键词：

neuroendocrine tumors; pancreas; gut; hereditary syndromes; multiple endocrine neoplasia type 1; neurofibromatosis; tuberous sclerosis complex; von Hippel-Lindau disease; gastrinoma; insulinoma; ENDOCRINE NEOPLASIA TYPE-1; HIPPEL-LINDAU-DISEASE; TUBEROUS SCLEROSIS COMPLEX; ISLET CELL TUMORS; CARCINOID-TUMORS; SUPPRESSOR GENE; MUTATIONAL ANALYSIS; DUODENAL SOMATOSTATINOMA; CONSENSUS CONFERENCE; FAMILIAL OCCURRENCE;

D O I：

10.1007/s00428-007-0450-3

中图分类号：

R36 [病理学];

学科分类号：

100104 ;

摘要：

Approximately 5-10% of neuroendocrine tumors (NETs) of the gastroenteropancreatic system (GEP) have a hereditary background. The known inherited syndromes include multiple endocrine neoplasia type 1, neurofibromatosis type 1, von Hippel-Lindau disease, and the tuberous sclerosis complex. This review discusses for each of these syndromes the: (1) involved genes and specific types of mutations, (2) disease prevalence and penetrance, (3) affected neuroendocrine tissues and related clinical syndromes, (4) special morphological features of NETs and their putative precursor lesions. In addition, GEP-NETs clustering in individual families or associated with other malignancies without known genetic background are discussed.

引用

页码：S29 / S38

页数：10

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