Mori Shoichi, Yoshioka H, Fukui T, Usami N, Uchiyama M, Sato N, Ito M, Kaji M, Ueda Y
Department of Thoracic Surgery, Nagoya University School of Medicine, Nagoya, Japan.
Kyobu Geka. 2005 Jul;58(7):597-601.
We report a case of Carney triad which is very rare disease composed of gastrointestinal stromal tumor (GIST), pulmonary chondroma and paraganglioma. A 15-year-old girl was reffered for treatment with multiple tumors in the left lung. At the age of 13, she underwent total gastrestomy for GIST. At that time multiple pulmonary tumors have already developed and made a diagnosis of chondroma. Progressive enlargement of their size and persistent bloody sputum made her received operation. Finally she underwent left pneumonectomy. In general all 3 tumors have manifested for a long time. Gastric tumors and paragangliomas are often lethal. This shows the necessity of intensive and long-term follow-up.
我们报告一例卡尼三联征病例,这是一种非常罕见的疾病,由胃肠道间质瘤(GIST)、肺软骨瘤和副神经节瘤组成。一名15岁女孩因左肺多发肿瘤前来接受治疗。13岁时,她因胃肠道间质瘤接受了全胃切除术。当时肺部已出现多个肿瘤,诊断为软骨瘤。肿瘤大小逐渐增大且持续咯血,促使她接受手术。最后她接受了左肺切除术。一般来说,所有这三种肿瘤都已存在很长时间。胃肿瘤和副神经节瘤往往是致命的。这表明进行强化和长期随访的必要性。