低度恶性纤维黏液样肉瘤3例临床病理分析  

Low-grade fibromyxoid sarcoma:a clinicopathologic analysis of three cases

作　　者：李娟[1] 陈易华[2] 胡晓松[1] 李淑蓉[1] 

机构地区：[1]成都医学院病理学教研室,成都610083 [2]成都军区总医院病理科,成都610083

出　　处：《诊断病理学杂志》2009年第3期221-223,共3页Chinese Journal of Diagnostic Pathology

摘　　要：目的探讨低度恶性纤维黏液样肉瘤（LGFMS）的临床病理学特征。方法对3例低度恶性纤维黏液样肉瘤的临床资料、病理形态学、免疫组织化学染色进行分析。结果3例均为男性,年龄18～43岁,平均31岁。肿瘤分别位于大腿、腹壁及前臂。最大径3～5 cm。镜下,肿瘤组织由少量形态温和的梭形细胞构成,间质胶原沉积,可见纤维区和不规则黏液区彼此移行。3例肿瘤细胞均vimentin（＋）,Ki-67〈2%,S-100、SMA、MSA、desmin、caldesmon、CD34和bcl-2均（-）。例1失访,例2和例3分别随访2年和1年均未见复发。结论低度恶性纤维黏液样肉瘤是一种少见的软组织肿瘤,具有良性组织学特点。鉴别诊断包括低度恶性黏液纤维肉瘤、黏液样脂肪肉瘤、纤维瘤病、黏液性神经纤维瘤、黏液样隆突性皮肤纤维肉瘤、炎性肌纤维母细胞瘤、黏液性恶性纤维组织细胞瘤等肿瘤。Objective To explore the clinicopathologic features of low-grade fibromyxoid sarcoma （LGFMS）. Methods Three cases of LGFMS were analyzed by clinical data, pathomorphology and immunohistochemistry. Results Among the 3 patients studied, all of they were males. Patients ranged in age from 18 to 43 years （mean 31）. The tumors were located in the thigh, the abdominal wall and the antebrachium. They measured from 3.0 to 5.0 cm （greatest diameter）. Histologically, the tumors were composed of a few bland spindle cell, with collagen deposition, pars fibrosa and irregularity myxoid district migration one another in matrix. Immunohistochemically, tumor cell were positive for vimentin, positive with Ki-67 were not so many as 2%, but negative for S-100, SMA, MSA, desmin, caldesmon, CD34 and bcl-2. In the following up 1 to 2 years, there was no relapse （case 1 lost follow-up）. Conclusion LGFMS is a rare soft tissue neoplasm with a deceptively benign histologic appearance. The following tumors must be included in the differential diagnosis： Low-grade myxofibrosareoma, myxoid liposarcoma, fibromatosis, myxoid neurofibroma, myxoid dermatofibrosarcoma protuberans, inflammatory myofibroblastic tumor and malignant fibrous histiocytomas.

关 键 词：纤维黏液样肉瘤 软组织 诊断 鉴别诊断 

分 类 号：R730.262[医药卫生—肿瘤]