Gastrointestinal stromal tumor risk factors

Gastrointestinal stromal tumor Microchapters
Home
Patient Information
Overview
Historical Perspective
Pathophysiology
Causes
Epidemiology and Demographics
Risk Factors
Screening
Differentiating Gastrointestinal stromal tumor from other Diseases
Natural History, Complications and Prognosis
Diagnosis
Staging
History and Symptoms
Physical Examination
Laboratory Findings
Chest X Ray
CT
MRI
Ultrasound
Other Imaging Findings
Other Diagnostic Studies
Treatment
Medical Therapy
Surgery
Primary Prevention
Secondary Prevention
Cost-Effectiveness of Therapy
Future or Investigational Therapies
Case Studies
Case #1
Gastrointestinal stromal tumor risk factors On the Web
Most recent articles
Most cited articles
Review articles
CME Programs
Powerpoint slides
Images
American Roentgen Ray Society Images of Gastrointestinal stromal tumor risk factors All Images X-rays Echo & Ultrasound CT Images MRI
Ongoing Trials at Clinical Trials.gov
US National Guidelines Clearinghouse
NICE Guidance
FDA on Gastrointestinal stromal tumor risk factors
CDC on Gastrointestinal stromal tumor risk factors
Gastrointestinal stromal tumor risk factors in the news
Blogs on Gastrointestinal stromal tumor risk factors
Directions to Hospitals Treating Gastrointestinal stromal tumor
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.