胸膜横纹肌肉瘤一例报道并文献复习

Pleura rhabdomyosarcoma: a case report and review of the literature

目的探讨原发性胸膜横纹肌肉瘤及肺内横纹肌肉瘤疾病的临床特点及诊治方案,以提高对该类疾病的认识。方法通过我们诊治的1例原发性胸膜横纹肌肉瘤病例,并行X线、PET-CT、胸、腹部多谱勒彩超及病理活组织检查,并检索近20年相似病例的文献,进行分析总结。结果双肺纹理增多,右侧胸腔中下份可见大片高密度影,右肺上叶可见絮状稍高密度模糊影。PET-CT显示:右侧胸膜见结节状、团块状软组织密度影,放射性摄取增高,纵膈内见增大淋巴结。胸水彩超检查及穿刺定位所见:右侧胸腔可探及一巨大的实质性肿块影像。病灶穿刺病理确诊为横纹肌肉瘤。结论原发性胸膜及肺内横纹肌肉瘤发病率极低,临床及影像学表现无特异性,胸片表现以肺部块影居多。易误诊为其他肺部肿瘤,其诊断主要依赖于病理学。在临床工作中应考虑到发病可能,进行鉴别诊断。

Objective Based on reported a case of pleura rhabdomyosarcoma and reviewed the above mentioned diseases in recent years,to summarize the clinical features,diagnosis and treatment of this disease for improve the understanding of this disease. Methods Reported a case of pleura rhabdomyosarcoma that we diagnosis and treatment,and retrieval of similar cases in 20 years in literature. Then summarizes the clinical features,diagnosis and treatment. Case： 65 year old male patient with right chest pain in 1 month,with irritating cough and a small amount of white mucus sputum,chest radiographs： the lung texture is increase,right pleural has a visible large high density shadow,right upper lung lobe shows flocculent slightly higher fuzzy density. PET- CT shows right pleura lesion has a nodular soft tissue density shadow,which has increased radioactivity. And some lymph nodes in Mediastinal are larger than normal. Color ultrasound examination of pleural effusion shows right pleura lesion detects a huge substantial tumor. Results The centesis biopsy was confirmed as rhabdomyosarcoma. Patient was not treated and leaves hospital after diagnosis. After a few days the disease progressed and the patient died at home,the direct cause of death is unknown. Conclusions The incidence rate of pleura rhabdomyosarcoma is very low; The clinical and imaging manifestations of pleura rhabdomyosarcoma are not specific. Chest radiographs are mainly performance as lung high density shadow. The diagnosis mainly depends on pathology,and is easy to be misdiagnosed as other common lung cancer. It should be paid enough attention to these diseases in clinical practice.