Moran C A, Suster S, Koss M N
Department of Pulmonary & Mediastinal Pathology, Armed Forces Institute of Pathology, Washington, D.C. 20306-6000, USA.
Histopathology. 1997 Feb;30(2):140-4. doi: 10.1046/j.1365-2559.1997.d01-583.x.
Two cases of malignant 'triton' tumour arising within lung parenchyma are described. The patients were a three-year-old child and a 53-year-old man. Both patients presented with shortness of breath and a large intrapulmonary mass on chest X-ray. Neither patient had a history of von Recklinhausen's neurofibromatosis. The lesions were treated by pneumonectomy. Grossly, both tumours presented as large, soft and gelatinous intraparenchymatous masses measuring 130 mm and 80 mm, respectively. Histologically, they were characterized by an atypical spindle cell proliferation embedded in an abundant myxoid stroma. Focal areas of rhabdomyoblastic differentiation characterized by large cells with abundant eosinophilic cytoplasm and occasional cytoplasmic cross-striations could be seen admixed with the atypical spindle cell elements. Immunohistochemical studies showed a focal positive reaction for S-100 protein in the atypical spindle cells embedded within the myxoid stroma, and a strong positive reaction for desmin and myoglobin in the rhabdomyoblastic areas. The child died three months after diagnosis with extension of the tumour into the thoracic cavity. The second patient has been lost to follow-up. Although rare, malignant 'triton' tumour should be considered in the differential diagnosis of primary spindle cell sarcomas of the lung.
本文描述了两例起源于肺实质的恶性“蝾螈”瘤。患者分别为一名3岁儿童和一名53岁男性。两名患者均表现为呼吸急促，胸部X线检查显示肺内有巨大肿块。两名患者均无冯雷克林霍增氏神经纤维瘤病病史。病变均通过肺切除术治疗。大体上，两个肿瘤均表现为大的、柔软的、胶冻状的实质内肿块，大小分别为130mm和80mm。组织学上，它们的特征是在丰富的黏液样基质中存在非典型梭形细胞增生。可见局灶性横纹肌母细胞分化区域，其特征为细胞大，胞质嗜酸性丰富，偶见胞质横纹，与非典型梭形细胞成分混合存在。免疫组织化学研究显示，黏液样基质内的非典型梭形细胞对S-100蛋白呈局灶阳性反应，横纹肌母细胞区域对结蛋白和肌红蛋白呈强阳性反应。该儿童在诊断后三个月因肿瘤扩展至胸腔而死亡。第二名患者失访。尽管罕见，但在肺原发性梭形细胞肉瘤的鉴别诊断中应考虑恶性“蝾螈”瘤。
