四肢软组织高度恶性血肿样肉瘤15例临床病理研究

Clinicopathologic Study of Hematoma-like Highly Malignant Sarcoma of Soft Tissues in the Extremities:A Report of 15 Cases

目的:探讨血肿样高度恶性肉瘤(hematoma鄄likehigh鄄gradesarcoma,HLHGS)的临床病理特点及预后。方法:分析15例HLHGS患者的临床、磁共振成像(MRI)和病理资料。标本经10%中性甲醛固定,石蜡包埋,苏木精鄄伊红染色和免疫组织化学ABC标记。结果:15例HLHGS临床均诊断为“血肿”。肿瘤位于大腿6例,小腿2例,上臂2例,踝部、腋窝、前臂、腹股沟和臀部各1例。MRI检查示:①病变长径与短径比为2∶1;②对比增强时显示“血肿壁”上肿瘤结节;③T1水平病变中间带强度同骨骼肌,T2水平中度增强,但强度低于液体。病理检查示病变中出血成分>85%,肿瘤组织学类型为恶性纤维组织细胞瘤7例,平滑肌肉瘤4例,滑膜肉瘤、恶性周围神经鞘膜瘤、横纹肌肉瘤和软组织黏液性软骨肉瘤各1例。按美国癌症联合会肿瘤分期Ⅲb12例,Ⅳb3例。术后随访6～60个月,其中无瘤生存6例,带瘤生存5例,死于肿瘤4例。复发率为13.3%,转移率为53.3%,2年生存率为73.3%。结论:血肿样肉瘤是高度侵袭性的软组织肉瘤,但其临床表现、超声波检查和CT扫描及细针抽吸活检易误诊为良性血肿。MRI检查可显示肿瘤性结节。CT引导下穿刺能明确诊断。

To study the clinicopathologic features and prognosis of hematoma-like highly malignant sarcoma(HLHMS)in the extremities.Methods:The clinical,MRI and pathologic data of15HLHMS patients treated in Washington Hospital Center were analyzed.Both Hematoxylin-and-Eosin and immunohistochemical ABC methods were used.Results:All the15cases of HLHMS were initially diagnosed as'hematoma'clinically.Six tumors were located in the thigh,2in the calf,2in the deltoid,and1in the elbow region,1in the axilla,1in the upper arm,1in the buttock,and1in the groin.The characteristics of HLHMS on MRI were:①The ratio of the diseased long axis to the short axis was2/1;②Contrast enhanced MR clearly revealed the tumor node on the'hematoma wall';③The diseased middle belt had the same intensity as that of the skeleton muscle at T 1 level and moderately enhanced at T 2 level but still lower than that of liquid.More than85%of tumor appeared as hemorrhagic lesion.Histopathologically,7tumors(46.7%)were malignant fibrous histiocytoma,4(26.7%)leiomyosarcoma,1synovial sarcoma,1malignant peripheral never sheath tumor,1rhabdomyosarcoma,and1extraskeletal myxoid chondrosarcoma.According to AJC(Amercian Joint Commission for Cancer Staging)staging system,12cases wereⅢb and3wereⅣb Post-operative follow-up data revealed that6patients were alive without evidence of disease;5patients survived with recurrent and/or metastasis tumor;4patients(26.7%)died of tumor month in6,13,17,and24of post-operation,respectively.Recurrence rate,metastasis rate and2-year survival rate were13.3%,53.3%and73.3%,respectively.Conclusion:HLHMS is a kind of tumor with highly invading,which is often diagnosed as hematoma in clinic.Ultrasound and fine needle aspiration have very low diagnostic accuracy,while MRI can avoid misdiagnois,CT-guided core biopsy is a very reliable method for HLHGS diagnosis.