Dual CD 117 Expression in Gastrointestinal Stromal Tumor (GIST) and Paraganglioma of Carney Triad: A Case Report

Abstract

Carney triad is a rare syndrome, with only 20 complete cases reported. We report a 36-year-old white woman with complete Carney triad, including metastatic gastric stromal tumor (GIST), pulmonary chondroma, and nonfunctioning extraadrenal paraganglioma. Immunohistochemistry was positive for CD34 and CD 117 (c-kit) in the GIST, and positive for chromogranin and CD 117 in the paraganglioma. Ultrastructural studies demonstrated skeinoid fibers in the GIST. To our knowledge, this is the 21st complete Carney triad case reported and the first report of dual expression CD 117 in both GIST and paraganglioma, a finding with intriguing pathogenetic implications related to the organization of the autonomic nervous system.

Keywords

Carney triad gastrointestinal stromal tumor (GIST)extra-adrenal paraganglioma pulmonary chondroma CD 117 (c-kit)autonomic nervous system

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