Suárez Antelo Juan, Rodríguez García Carlota, Montero Martínez Carmen, Verea Hernando Héctor
Servicio de Neumología, Complejo Hospitalario Universitario, A Coruña, Spain.
Arch Bronconeumol. 2010 Jan;46(1):44-6. doi: 10.1016/j.arbres.2009.03.008. Epub 2009 Aug 4.
Primary thoracic sarcomas are very rare. The most common intrathoracic variants are synovial sarcoma, angiosarcoma, leiomyosarcoma, rhabdomyosarcoma, and sarcomatoid mesothelioma. Although thoracic Ewing sarcoma/primitive neuroectodermal tumor (PNET) usually develops on the chest wall, there have been reports of primary Ewing sarcoma/PNET of the lung. We present the case of a 22-year-old woman with Ewing sarcoma/PNET diagnosed following histologic, immunohistochemical, and in situ hybridization studies of a bronchial biopsy specimen. Radiography, ventilation-perfusion scintigraphy, and a bone marrow biopsy confirmed that the tumor was not metastatic. The patient was started on a chemotherapy regimen of vincristine, actinomycin, cyclophosphamide, doxorubicin, ifosfamide, and etoposide and responded well. She is now being seen regularly at our outpatient clinic.
原发性胸段肉瘤非常罕见。最常见的胸内变异型包括滑膜肉瘤、血管肉瘤、平滑肌肉瘤、横纹肌肉瘤和肉瘤样间皮瘤。尽管胸段尤因肉瘤/原始神经外胚层肿瘤（PNET）通常发生于胸壁，但也有原发性肺尤因肉瘤/PNET的报道。我们报告一例22岁女性，经支气管活检标本的组织学、免疫组化和原位杂交研究确诊为尤因肉瘤/PNET。X线摄影、通气灌注闪烁扫描和骨髓活检证实肿瘤无转移。该患者开始接受长春新碱、放线菌素、环磷酰胺、阿霉素、异环磷酰胺和依托泊苷的化疗方案，反应良好。她现在定期在我们的门诊就诊。
