Hereditary neuroendocrine tumors of the gastroenteropancreatic system

Martin Anlauf; Nele Garbrecht; Juliane Bauersfeld; Anja Schmitt; Tobias Henopp; Paul Komminoth; Philipp U. Heitz; Aurel Perren; Günter Klöppel

doi:10.1007/s00428-007-0450-3

Hereditary neuroendocrine tumors of the gastroenteropancreatic system

Martin Anlauf, Nele Garbrecht, Juliane Bauersfeld, Anja Schmitt, Tobias Henopp, Paul Komminoth, Philipp U. Heitz, Aurel Perren, Günter Klöppel

Research output: Contribution to journal › Review article › peer-review

97 Scopus citations

Abstract

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.

Original language	English
Pages (from-to)	S29-S38
Journal	Virchows Archiv
Volume	451
Issue number	SUPPL. 1
DOIs	https://doi.org/10.1007/s00428-007-0450-3
State	Published - Aug 2007
Externally published	Yes

Keywords

Gastrinoma
Gut
Hereditary syndromes
Insulinoma
Multiple endocrine neoplasia type 1
Neuroendocrine tumors
Neurofibromatosis
Pancreas
Tuberous sclerosis complex
Von Hippel-Lindau disease

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10.1007/s00428-007-0450-3

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title = "Hereditary neuroendocrine tumors of the gastroenteropancreatic system",

abstract = "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.",

keywords = "Gastrinoma, Gut, Hereditary syndromes, Insulinoma, Multiple endocrine neoplasia type 1, Neuroendocrine tumors, Neurofibromatosis, Pancreas, Tuberous sclerosis complex, Von Hippel-Lindau disease",

author = "Martin Anlauf and Nele Garbrecht and Juliane Bauersfeld and Anja Schmitt and Tobias Henopp and Paul Komminoth and Heitz, {Philipp U.} and Aurel Perren and G{\"u}nter Kl{\"o}ppel",

note = "Funding Information: Acknowledgment This paper was supported by Hensel Stiftung, Kiel, Germany (F370011; MA and GK), Swiss National Foundation (SNF 31-108257; AP and PK). Nele Garbrecht and Tobias Henopp have a fellowship sponsored by Ipsen GmbH, Ettlingen, and Hensel-Stiftung, Kiel, Germany.",

year = "2007",

month = aug,

doi = "10.1007/s00428-007-0450-3",

language = "English",

volume = "451",

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journal = "Virchows Archiv",

issn = "0945-6317",

number = "SUPPL. 1",

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TY - JOUR

T1 - Hereditary neuroendocrine tumors of the gastroenteropancreatic system

AU - Anlauf, Martin

AU - Garbrecht, Nele

AU - Bauersfeld, Juliane

AU - Schmitt, Anja

AU - Henopp, Tobias

AU - Komminoth, Paul

AU - Heitz, Philipp U.

AU - Perren, Aurel

AU - Klöppel, Günter

N1 - Funding Information: Acknowledgment This paper was supported by Hensel Stiftung, Kiel, Germany (F370011; MA and GK), Swiss National Foundation (SNF 31-108257; AP and PK). Nele Garbrecht and Tobias Henopp have a fellowship sponsored by Ipsen GmbH, Ettlingen, and Hensel-Stiftung, Kiel, Germany.

PY - 2007/8

Y1 - 2007/8

N2 - 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.

AB - 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.

KW - Gastrinoma

KW - Gut

KW - Hereditary syndromes

KW - Insulinoma

KW - Multiple endocrine neoplasia type 1

KW - Neuroendocrine tumors

KW - Neurofibromatosis

KW - Pancreas

KW - Tuberous sclerosis complex

KW - Von Hippel-Lindau disease

UR - http://www.scopus.com/inward/record.url?scp=34548018958&partnerID=8YFLogxK

U2 - 10.1007/s00428-007-0450-3

DO - 10.1007/s00428-007-0450-3

M3 - Review article

C2 - 17684762

AN - SCOPUS:34548018958

SN - 0945-6317

VL - 451

SP - S29-S38

JO - Virchows Archiv

JF - Virchows Archiv

IS - SUPPL. 1

ER -

Hereditary neuroendocrine tumors of the gastroenteropancreatic system

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Keywords

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