Department of Radiology, Children's University Hospital, Toulouse, France.
Pediatr Radiol. 2012 May;42(5):624-6. doi: 10.1007/s00247-011-2241-x. Epub 2011 Oct 14.
We report a rare example of anaemia and hypertension due to an incomplete Carney triad in a 14-year-old girl with no previous medical history. This rare non-familial syndrome generally involves two disparate tumours: gastrointestinal stromal tumour, paraganglioma and/or pulmonary chondroma. The complete triad is a syndrome that involves at least five loci: stomach, oeophagus, lung, the paraganglionic system, adrenal (cortex or medulla). The pathogenesis is unclear as these tumours have different embryological origins. Surgical treatment is necessary, and long-term follow-up is advisable as patients with Carney triad may re-present with tumour(s), even several years after the first presentation.
我们报告了一例罕见的 14 岁女孩因不完全卡尼三联征引起的贫血和高血压,该患者无既往病史。这种罕见的非家族性综合征通常涉及两种不同的肿瘤:胃肠道间质瘤、副神经节瘤和/或肺软骨瘤。完全三联征是一种涉及至少五个部位的综合征:胃、食管、肺、副神经节系统、肾上腺(皮质或髓质)。其发病机制尚不清楚,因为这些肿瘤具有不同的胚胎起源。需要进行手术治疗,并且建议进行长期随访,因为即使在首次出现后的几年,卡尼三联征患者也可能会再次出现肿瘤。