Przygodzki R M, Moran C A, Suster S, Koss M N
Department of Pulmonary, Armed Forces Institute of Pathology, Washington, DC, USA.
Mod Pathol. 1995 Aug;8(6):658-61.
Three cases of primary pulmonary rhabdomyosarcoma in adults are presented. The patients were all men between the ages of 57 and 78 yr (mean 67.5). All patients presented with symptoms referable to their tumors, including cough, shortness of breath, pleuritic chest pain, and weight loss. In one patient, a history of tobacco and alcohol abuse was obtained. Anatomically, two tumors were located in the left upper lobe and one in the left lower lobe. Grossly, the tumors ranged in size from 6 to 11.5 cm and were tan-gray, firm masses with areas of necrosis and hemorrhage. Histologically, the tumors were characterized by a spindle cell proliferation admixed with areas showing a pleomorphic cell population with numerous rhabdomyoblasts and areas of hemorrhage and necrosis. Immunohistochemically, all three tumors showed strong positivity with desmin and myoglobin antibodies and negative staining with antibodies against keratin, epithelial membrane antigen, and S-100 protein. All patients had a fatal outcome. Two patients died a few days after admission with respiratory distress; the third one died 2 years after diagnosis with widely metastatic disease. Autopsy findings in all cases disclosed disseminated metastases to multiple abdominal and thoracic organs. Primary pulmonary rhabdomyosarcoma should be considered in the differential diagnosis of poorly differentiated pulmonary neoplasms in adults and should be distinguished from other primary and metastatic sarcomas.
本文报告了3例成人原发性肺横纹肌肉瘤。患者均为男性，年龄在57至78岁之间（平均67.5岁）。所有患者均表现出与肿瘤相关的症状，包括咳嗽、气短、胸膜炎性胸痛和体重减轻。其中1例患者有吸烟和酗酒史。从解剖学上看，2个肿瘤位于左上叶，1个位于左下叶。大体上，肿瘤大小为6至11.5 cm，呈棕灰色，质地坚硬，伴有坏死和出血区域。组织学上，肿瘤的特征是梭形细胞增生，伴有多形性细胞群区域，有大量横纹肌母细胞以及出血和坏死区域。免疫组化方面，所有3个肿瘤对结蛋白和肌红蛋白抗体呈强阳性，而对角蛋白、上皮膜抗原和S-100蛋白抗体呈阴性染色。所有患者均预后不良。2例患者在入院后数天因呼吸窘迫死亡；第3例患者在诊断为广泛转移性疾病后2年死亡。所有病例的尸检结果均显示肿瘤已扩散转移至多个腹部和胸部器官。在成人低分化肺肿瘤的鉴别诊断中应考虑原发性肺横纹肌肉瘤，并应与其他原发性和转移性肉瘤相鉴别。
