巨大原发性纵隔滑膜肉瘤经新辅助化疗治疗的惊人结果。
The surprising outcome of a giant primary mediastinal synovial sarcoma treated with neoadjuvant chemotherapy.
机构信息
Division of Thoracic Surgery, State University of Rio de Janeiro, Rio de Janeiro, Brazil;
出版信息
J Thorac Dis. 2013 Feb;5(1):94-6. doi: 10.3978/j.issn.2072-1439.2012.12.05.
There are only a few cases of primary mediastinal synovial sarcoma in the literature. Normally, they do not respond well to chemotherapy. In our case, a 30-year-old patient was admitted due to thoracic pain, dyspnea, orthopnea, cough, hoarseness and weight loss over a 3-month period as well as a dramatic worsening a week before the admission. A chest radiography showed a completely white left hemithorax and contralateral mediastinal shift; in addition, a chest tomography revealed a giant heterogeneous mediastinal mass, lung atelectasia and a small pleural effusion. The patient was submitted to Chamberlain procedure (biopsy) under local anesthesia and the diagnosis of a synovial sarcoma was obtained after immunohistochemical analysis. Due to his poor general condition, he received chemotherapy first, with a dramatic response, after what, the mass that had been reduced was removed surgically. After a 5-year- follow-up period there are no signs of disease recurrence.
文献中仅有少数原发性纵隔滑膜肉瘤的病例。通常，它们对化疗的反应不佳。在我们的病例中，一名 30 岁的患者因胸痛、呼吸困难、端坐呼吸、咳嗽、声音嘶哑和体重减轻 3 个月，并在入院前一周急剧恶化而入院。胸部 X 线片显示左侧整个胸腔完全变白，对侧纵隔移位；此外，胸部 CT 显示巨大的异质性纵隔肿块、肺不张和少量胸腔积液。患者在局部麻醉下接受了 Chamberlain 手术（活检），并通过免疫组织化学分析得出滑膜肉瘤的诊断。由于他的一般状况不佳，他首先接受了化疗，反应非常明显，之后，肿块缩小后进行了手术切除。经过 5 年的随访，没有疾病复发的迹象。
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