Gastrointestinal stromal tumor risk factors: Difference between revisions
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* Age-GISTs are more common in people aged 50 to 80. | * Age-GISTs are more common in people aged 50 to 80. | ||
*Genetic syndromes: | *Genetic syndromes: | ||
:*[[Neurofibromatosis]] type 1 (or von Recklinghausen disease). | :*[[Neurofibromatosis]] type 1 (or von Recklinghausen disease). It is an autosomal dominant condition caused by muatation in NF-1 gene. Patients with neurofibromatosis type 1 develop GIST as multiple tumors in the small intestine. | ||
:*Carney-Stratakis syndrome- | :*Carney-Stratakis syndrome- It is a rare, autosomal dominant condition due to muatation in succinate dehydrogenase enzyme complex. Patients with Carney-Stratakis syndrome have an increased risk of GISTs and paragangliomas. Patients are generally in their second decade of life and more likely to have multiple GISTs. | ||
:*Familial gastrointestinal stromal tumor syndrome- It is a rare, | :*Familial gastrointestinal stromal tumor syndrome- It is a rare, autosomal dominant condition that leads to an increased risk of developing GISTs. These patients have a mutation in their KIT gene (90% cases) or PDGFRA gene (5-10% cases). Patients with familial GIST syndrome develop GIST at an early age and are more likely to have multiple GISTs. | ||
==References== | ==References== | ||
Revision as of 01:59, 8 December 2017
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Gastrointestinal stromal tumor Microchapters |
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Differentiating Gastrointestinal stromal tumor from other Diseases |
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Gastrointestinal stromal tumor risk factors On the Web |
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American Roentgen Ray Society Images of Gastrointestinal stromal tumor risk factors |
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Risk calculators and risk factors for Gastrointestinal stromal tumor risk factors |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Parminder Dhingra, M.D. [2]
Overview
The most potent risk factor in the development of GISTs are age 50-80 and certain genetic syndromes like neurofibromatosis type 1, Carney-Stratakis syndrome and familial gastrointestinal stromal tumor syndrome.
Risk factors
- Age-GISTs are more common in people aged 50 to 80.
- Genetic syndromes:
- Neurofibromatosis type 1 (or von Recklinghausen disease). It is an autosomal dominant condition caused by muatation in NF-1 gene. Patients with neurofibromatosis type 1 develop GIST as multiple tumors in the small intestine.
- Carney-Stratakis syndrome- It is a rare, autosomal dominant condition due to muatation in succinate dehydrogenase enzyme complex. Patients with Carney-Stratakis syndrome have an increased risk of GISTs and paragangliomas. Patients are generally in their second decade of life and more likely to have multiple GISTs.
- Familial gastrointestinal stromal tumor syndrome- It is a rare, autosomal dominant condition that leads to an increased risk of developing GISTs. These patients have a mutation in their KIT gene (90% cases) or PDGFRA gene (5-10% cases). Patients with familial GIST syndrome develop GIST at an early age and are more likely to have multiple GISTs.
References