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恶性纤维性组织细胞瘤(MFH)是成人常见的软组织癌肿,儿童罕见。MFH起源于深筋膜,常发生于肢体和后腹膜。迄今,仅见两例原发于成人肺部的报告,本文作者报告一例原发于儿童肺部的MFH。病例女性,12岁。主诉呼吸和活动时胸痛。体检和实验室检查无异常发现。胸片示右上肺有8.5×9.0cm肿块影。CT扫描见右上肺有一密度不等的大肿块,肿块中央有提示组织坏死的低密度区,其间有散在点状钙化影。右上肺叶支气管被肿块包绕,提示肿块位于肺内。肿块与纵膈分界清楚。行右上肺叶切除术,肿块大小为11×10×12cm,重230g。肿瘤已部分坏死,外表皇粘液样外观。病理检查呈MFH的组织特征。手术5个月后,先后出现两肺和脑部的转移性病灶。
Malignant fibrous histiocytoma (MFH) is an adult common soft tissue cancer, rare in children. MFH originated in the deep fascia, often occurs in the limbs and retroperitoneum. To date, only two reports of primary lung cancer have been reported, and the authors report an example of MFH originating in the lungs of children. Case female, 12 years old. Chest pain complains of breathing and activity. Physical examination and laboratory tests found no abnormalities. Chest radiograph showed a right upper lung 8.5 × 9.0cm mass. CT scan see the right upper lung has a density of large lumps, lumps prompted the central tissue necrosis of the low density area, during which there are scattered spots of calcification. The upper right lobe bronchi is surrounded by a mass, suggesting that the mass is in the lung. Mass and mediastinal clear boundaries. Right upper lobectomy, mass size of 11 × 10 × 12cm, weight 230g. The tumor has been partially necrotic, the appearance of the appearance of the emperor mucus. Pathological examination was MFH tissue characteristics. Five months after surgery, two lung and brain metastatic lesions successively appeared.