Like adult GIST, pediatric GIST can be further divided into subgroups. The most familiar and perhaps the most common type does not seem to have a distinct name so let’s just call it “Pediatric GIST.” It most commonly affects girls between the ages of about 6 to 18 and almost always starts in the stomach.
The second well-known type of GIST affecting young people is called “Carney’s Triad.” It is named after Dr. J. Aidan Carney who first described it in 1977. Patients with Carney’s Triad may have several different types of tumors including GIST, pulmonary chondroma, and/or functioning extra-adrenal paraganglioma. If any two of these tumors are present, a diagnosis of the “triad” can be made, particularly if age and sex factors are supportive.
In 2002, Dr. Carney and Dr. Constantine Stratakis reported on a new syndrome that was similar to, yet distinct, from Carney’s triad. They had found 12 patients in 5 families with two parts of the “triad,” paraganglioma and GIST. Since this condition appeared to be inheritable and the “true Carney’s Triad” did not, Carney and Stratakis concluded that this syndrome was different than Carney’s Triad and called it “Familial Paraganglioma and Gastric Stromal Sarcoma” (also known as Carney-Stratakis Syndrome). The patients varied in age from 9 to 46 years old at diagnosis with 9 of the 14 patients under the age of 23.
When looking at multiple medical reports, it appears like there may be one more type of pediatric GIST. The youngest GIST patients of all may be born with GIST and need surgery soon after birth. This type of GIST, called “neonatal or congenital GIST”, appears to affect both boys and girls (although girls are more common) and seems to start in the intestines instead of the stomach. We found 6 cases described as GIST in newborns, however there seems to be some doubt about whether or not these are really leiomyosarcomas or GISTs.
Patient Disease and Characteristics by Age Group at Diagnosis
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Resources for children created by pediatric GIST survivor, Erin MacBean (see Erin’s story).
What is Cancer?
What is GIST?
The Digestive System
Next Steps:
The Science and Biology of Pediatric GIST
Wildtype GIST
Information on Mutations in GIST and Mutational Testing
Newly Diagnosed Patients
Join the Life Raft Group and the GIST Collaborative Tissue Bank
Contribute to Real World Evidence working toward a cure!
To join the Pediatric GIST and Carney’s Triad listserv, please visit GIST Support International
This pediatric portion of the website is dedicated to the memory of Jonathan Montague.
Articles on Pediatric GIST
Life Fest Pediatric/SDH Deficient GIST Track Provides Wealth of Information
The 2018 Life Fest Meeting was held in Miami on July 13 - 15 and was attended by 140 patients, friends, family and caregivers. This was a truly international event, with participation from 20 [...]
NIH Clinic Combines Research, Knowledge, and Hope
I had the pleasure of attending the 15th Pediatric and Wildtype Clinic along with Becky Owens from GIST Support International as patient advocates. The Clinic was held at the National Institute of Health in [...]
15th Pediatric and Wildtype Clinic Provides Support and Education
The 15th Pediatric and Wildtype GIST Clinic was recently held at the National Institute of Health in Bethesda, Maryland on July 5 - 7, 2017. The Clinic is a collaborative effort between clinicians and [...]
14th NIH Pediatric and Wildtype GIST Clinic Convenes
Sara Rothschild (LRG), Erin MacBean (LRG), Phyllis Gay (GSI) and Becky Besenhaver (GSI) By Rebecca Bensenhaver, GIST Support International RepresentativeThe 14th Pediatric and Wildtype GIST Clinic was held at the National Institute of Health [...]
On the road with Milly: Toto, we’re not in D.C. anymore!
Hi friends, It's time to begin the second leg of this super trip. So with a heavy heart and a heavier suitcase we bid goodbye to OVAC Lobby Day and Washington D.C. That's right- we! I [...]
Developing GISTKids.org
During the Life Raft Group Life Fest 2014, a panel of young GIST patients and family members gathered around a table to discuss ideas for advocating for SDH-deficient and wild type GIST. The group agreed [...]
