Gastrointestinal Stromal Tumor (GIST)

Carney-Triad

High incidence of metastasis and recurrence but course is protracted
- Reported 100% 10 year survival and 73% 40 year survival (Zhang)
Presentation (except for the triad features), pathology and behavior are essentially the same as Carney-Stratakis syndrome and sporadic SDHB deficient (pediatric type) GIST
- Rule out Carney-Stratakis syndrome
  - Familial, associated with paragangliomas but no chondromas

References (see left side bar for general GIST bibliography)
- Agaimy A, Pelz AF, Corless CL, Wünsch PH, Heinrich MC, Hofstaedter F, Dietmaier W, Blanke CD, Wieacker P, Roessner A, Hartmann A, Schneider-Stock R. Epithelioid gastric stromal tumours of the antrum in young females with the Carney triad: a report of three new cases with mutational analysis and comparative genomic hybridization. Oncol Rep. 2007 Jul;18(1):9-15.
- Zhang L, Smyrk TC, Young WF Jr, Stratakis CA, Carney JA. Gastric stromal tumors in Carney triad are different clinically, pathologically, and behaviorally from sporadic gastric gastrointestinal stromal tumors: findings in 104 cases. Am J Surg Pathol. 2010 Jan;34(1):53-64.
- Gaal J, Stratakis CA, Carney JA, Ball ER, Korpershoek E, Lodish MB, Levy I, Xekouki P, van Nederveen FH, den Bakker MA, O'Sullivan M, Dinjens WN, de Krijger RR. SDHB immunohistochemistry: a useful tool in the diagnosis of Carney-Stratakis and Carney triad gastrointestinal stromal tumors. Mod Pathol. 2011 Jan;24(1):147-51