摘要目的 分析肉瘤样恶性间皮瘤(sarcomatoid malignant mesothelioma,SMM)的临床病理学特征,探讨SMM的诊断要点及其鉴别诊断.方法 回顾性复习2009年1月至2013年9月收集的22例SMM的临床资料、病理学形态和免疫表型,分析预后资料.结果 22例患者的平均年龄54岁(范围24～ 73岁),男女比例为1.0∶1.2.多数患者无明确的石棉接触史.14例发生于胸膜,8例位于腹膜.临床上,发生于胸膜者多因咳嗽、气喘和胸痛就诊,发生于腹膜者多有恶心、腹胀和腹痛等症状.影像学显示,多数病例(8/11)表现为胸壁或腹腔内大小不等的结节状肿块,仅少数病例(3/11)表现为胸膜弥漫性增厚.所有病例均无肺或腹腔实质脏器内占位性病变.组织学上,19例呈纤维肉瘤样或未分化多形性肉瘤样,2例为促结缔组织增生性,1例伴有骨肉瘤样分化.免疫组织化学检测显示,所有病例均表达广谱细胞角蛋白(AE1/AE3),多数病例尚表达D2-40 (15/20),部分病例弱阳性或灶性表达钙视网膜蛋白(9/21)和WT1(10/14),不表达甲状腺转录因子-1、Napsin A、肺表面活性物质相关蛋白A、p63和CD34等.随访11例(1～36个月),6例带瘤生存,1例复发,4例死亡.中位生存时间5个月(平均8个月).结论 SMM的诊断必需结合临床、影像学和病理学资料.广谱细胞角蛋白联合D2-40标记对SMM的诊断具有提示作用,而钙视网膜蛋白仅在部分SMM病例中有弱阳性表达,WT1常为灶性表达.诊断SMM之前还需注意除外肉瘤样癌、滑膜肉瘤、孤立性纤维性肿瘤和纤维性胸膜炎等具有相似形态的梭形细胞肿瘤.

abstractsObjective To elaborate on the clinical and pathologic features of sarcomatoid malignant mesothelioma (SMM),its diagnostic criteria and differential diagnoses.Methods Twenty-two cases of SMM retrieved from in-house and consultation files (between January 2009 to September 2013) were reviewed with emphasis on the clinicopathologic characteristics,immunophenolypes and the prognostic impact.Results The mean age of the patients was 54 years (ranged from 24-73 years).There was no sexual predilection and the majorily of the patients did not have history-of asbestos exposure.Overall,14 tumors developed in the pleura and 8 cases arose from the peritoneal cavity-.Clinically,patients presented signs and symptoms in accord with the location of the tumors,notably coughing,shortness of breath,and chest pain for patients with pleural origin,and nausea,abdominal distention and abdominal pain for those with peritoneal primary-.In most cases,CT and MRI scan demonstrated lobulated masses (8/11).However,diffuse infiltrative growth patterns were observed exclusively in a minority of pleural cases (3/11).No visceral lesion was observed in any-case.Histologically,19 cases had either fibrosarcomatous or undifferentiated pleomorphic sarcoma-like appearance.Two cases were consistent with desmoplastic mesothelioma.One case contained osteosarcomatous element.All cases expressed pan-cytokeratin (AE1/AE3),and most cases were also positive for D2-40 (15/20).The staining of calretinin (9/21) and WT1 (10/14) was generally weak and focal.They were all negative for TTF-1,napsin A,SP-A,p63 and CD34.Follow-up information (range from 1 to 36 months) was available in 11 cases,6 of which were alive with unresectable tumor,1 patient with recurrent disease and 4 patients succumbed to disease.The overall survival was 5 months (mean 8 months).Conclusions The diagnosis of SMM is achieved by comprehensive evaluation of medical history,imageological and pathological findings.Since calretinin immunoreactivity is infrequently observed in SMM,application of pan-cytokeratin and D2-40 immunostains offers a reasonable alternative for diagnosis.Diagnosis of SMM can be made by excluding a variety of spindle cell neoplasms with overlapping features,such as sarcomatoid carcinoma,synovial sarcoma,solitary fibrous tumor and fibrous pleuritis.