Carney's Triad

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More about the different types of pediatric GIST - Pediatric GIST | Carney's Triad

Carney's Triad

The second well-known type of GIST affecting young people is called “Carney’s Triad.” It is named after Dr. P. 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. It is important that patients less than 35 years of age who have any one of the three tumors be examined periodically in search of the others according to Dr. Carney.

By 1983, 24 cases of Carney's Triad had been reported and, by 1999, 79 cases had been reported. Carney’s triad affects mostly younger females but there is one report of an 84-year-old man who was found to have Carney’s Triad during an autopsy.

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” (GIST is sometimes called “Gastric Stromal Sarcoma”). The patients varied in age from 9 to 46 years old at diagnosis with 9 of the 14 patients under the age of 23.

This pediatric portion of the website is dedicated to the memory of Jonathan Montague.

1. Diagnosis of Gastrointestinal Stromal Tumors: A Consensus Approach-Christopher D. M. Fletcher, MD, FRCPATH, Jules J. Berman, MD, PhD, Christopher Corless, MD, PhD, Fred Gorstein, MD, Jerzy Lasota, MD, PhD, B. Jack Longley, MD, Markku Miettinen, MD, Timothy J. O'Leary, MD, PhD, Helen Remotti, MD, Brian P. Rubin, MD, Phd, Barry Shmookler, MD, Leslie H. Sobin, MD, and Sharon W. Weiss, MD

2. Gastrointestinal Stromal Tumors of the Stomach. A Clinicopathologic, Immunohistochemical, and Molecular Genetic Study of 1765 Cases With Long-term Follow-up. American Journal of Surgical Pathology. 29(1):52-68, January 2005.
Miettinen, Markku MD *; Sobin, Leslie H MD +; Lasota, Jerzy MD

3. Prognostic Value of KIT Mutation Type, Mitotic Activity, and Histologic Subtype in Gastrointestinal Stromal Tumors. By Samuel Singer, Brian P. Rubin, Marcia L. Lux, Chang-Jie Chen, George D. Demetri, Christopher D. M. Fletcher, and Jonathon A. Fletcher

4. A New Observation of the Carney's Triad with Long Follow-Up Period and Additional Tumors

Lucio Scopsi, M.D.,Paola Collini, M.D., Giuseppe Muscolino, M.D.