Carney's triad, a rare disorder affecting young females, is characteri
zed by the presence of at least two of the three following neoplasms:
gastric epithelioid leiomyosarcoma, extra-adrenal paraganglioma, and p
ulmonary chondroma. Diagnosis and follow-up evaluation of cases of Car
ney's triad may require an approach that uses a combination of modalit
ies, including magnetic resonance imaging (MRI), metaiodobenzyl-guanid
ine (MIBG)I 131 scintigraphy, and computed tomography. MRI is as effec
tive 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 modal
ity 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.