Abstract

ABSTRACTCarney's triad, a rare disorder affecting young females, is characterized by the presence of at least two of the three following neoplasms: gastric epithelioid leiomyosarcoma, extra-adrenal paraganglioma, and pulmonary chondroma. Diagnosis and follow-up evaluation of cases of Carney's triad may require an approach that uses a combination of modalities, including magnetic resonance imaging (MRI), metaiodo-benzyl-guanidine (MIBG) I 131 scintigraphy, and computed tomography. MRI is as effective as MIBG scintigraphy in detecting functioning paragangliomas. In the evaluation of suspected cases of Carney's triad in which there is clinical and biochemical evidence of a paraganglioma, MRI is the modality of choice for screening and follow-up. We report a case of gastric leiomyosarcoma and extra-adrenal paraganglioma in a young woman whose initial presentation was at 7 years of age. To our knowledge, this is the earliest presentation of this disorder.