Erb R E, Delbeke D, Falke T H, Sandler M P
Vanderbilt University Medical Center, Department of Radiology, Nashville, TN 37232-2675.
South Med J. 1994 Sep;87(9):951-5. doi: 10.1097/00007611-199409000-00020.
Carney'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), metaiodobenzyl-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.
卡尼三联征是一种影响年轻女性的罕见疾病,其特征是存在以下三种肿瘤中的至少两种:胃上皮样平滑肌肉瘤、肾上腺外副神经节瘤和肺软骨瘤。卡尼三联征病例的诊断和随访评估可能需要采用多种检查方法相结合的方式,包括磁共振成像(MRI)、间碘苄胍(MIBG)I 131闪烁扫描和计算机断层扫描。MRI在检测功能性副神经节瘤方面与MIBG闪烁扫描效果相当。在评估疑似卡尼三联征且有副神经节瘤临床和生化证据的病例时,MRI是筛查和随访的首选检查方法。我们报告了一例年轻女性胃平滑肌肉瘤和肾上腺外副神经节瘤病例,该患者最初发病于7岁。据我们所知,这是该疾病最早的发病表现。