原发性胸膜软骨肉瘤致血性胸腔积液一例并文献复习

Hemothorax caused by primary pleural chondrosarcoma： a case report and review of literature

目的 分析原发性胸膜软骨肉瘤致血性胸腔积液的临床特征,提高医生对原发性胸膜软骨肉瘤的认识.方法 回顾性分析1例2015年9月确诊的原发性胸膜软骨肉瘤患者的临床特征,并结合文献进行复习.以“胸膜,软骨肉瘤”及“pleura,chondrosarcoma”为关键词检索1980年1月至2015年10月万方数据库、中国知网及PubMed数据库的相关文献,共获得文献6篇,其中中文1篇,英文5篇.结果 男,29岁,因“发热、胸闷、气促20 d”就诊.临床症状为发热、胸闷、气促.胸部CT示右第4后肋处骨性肿块,右侧大量胸腔积液,常规检查提示血性胸腔积液.开胸手术见右侧血性胸腔积液,胸膜顶骨性肿块.行右侧胸壁肿块切除＋胸膜纤维板剥离术.术后病理诊断为高分化软骨肉瘤.随访至今未复发.文献检索报道了6例原发性胸膜软骨肉瘤,其中临床资料完整的5例,男2例,女3例,年龄28 ～ 70岁;资料不全的1例,为78岁男性,均为个案报道.结论 胸膜原发性软骨肉瘤罕见,而以血性胸腔积液为临床表现的更罕见,临床表现缺乏特异性.该病易漏诊或误诊,依靠病理活检确诊,手术治疗是其最有效的治疗方法.

Objective To analyze the clinical features of one case of spontaneous hemothorax caused by primary pleural chondrosarcoma and therefore to improve the understanding of this disease.Methods The clinical features of a case with primary pleural chondrosarcoma were analyzed retrospectively and the related literatures were reviewed.The literature review was carried out with ＆quot;primary pleural,chondrosarcoma＆quot; in Chinese and English respectively,as the search terms in Wanfang Data,CNKI and PubMed database from January 1980 to October 2015.A total of 6 articales,1 in Chinese and 5 in English,were reviewed.Results A 29 year-old male patient was admitted to the hospital because of fever,chest tightness,shortness of breath for 20 days.CT scan of the chest showed a mass near the right posterior fourth rib and right pleural effusion.Routine examination of the pleural effusion confirmed the presence of hemothorax.Thoracotomy was performed and revealed hemothorax in the right thorax,and a mass near the pleural apex.The tumor was removed by surgery and pleural decortication was also performed.Pathology study confirmed the diagnosis of high-differentiated chondrosarcoma.The patient was followed and there was no recurrence until now.A total of 6 case reports were retrieved from Wanfang Data,CNKI and PubMed.Five cases had complete data,including 2 males and 3 females（age from 28 to70）,and another （a 78-year old male） without adequate data.Conclusions Primary pleural chondrosarcoma is a rare disease,and hemothorax as the first manifestation is even rare.It is easily to be misdiagnosed due to nonspecific clinical symptoms.The final diagnosis depends ultimately on pathological biopsy.Thoracotomy is the most effective method for treatment of primary pleural chondrosarcoma.