发生于皮肤的Langerhans细胞肉瘤一例  

Cutaneous Langerhans cell sarcoma： a ease report

作　　者：王长松[1] 印剑[1] 原旭涛[1] 王仰坤[1] 陈燕平[2] 

机构地区：[1]解放军第150中心医院病理科,河南洛阳471031 [2]解放军第150中心医院检验科,河南洛阳471031

出　　处：《中华皮肤科杂志》2009年第10期685-687,共3页Chinese Journal of Dermatology

摘　　要：患者男，41岁。双侧腹股沟及左腰部肿块半年余，破溃2周。包块初起质软，逐渐增大变硬，疼痛。近2周包块表面出现破溃。血常规正常，血糖偏高。给予抗生素治疗，包块仍红肿，术中见包块位于皮下，与周围界限较清楚，但黏连严重，肿块内部分区域坏死。病理检查示真皮及皮下组织中肿瘤细胞弥漫成片排列，大部分细胞体积较大，细胞形态多样；胞浆较丰富、嗜酸性；胞核不规则形，较大，呈分叶状或有凹痕，有的可见一纵向核沟，核仁突出，可见大量多核巨细胞。免疫组化：瘤细胞S-100蛋白、CD1a和波形蛋白阳性，溶菌酶弱阳性，CD68部分细胞阳性；CD20、CD3、CD30、CKpan、Actin、EMA、AFP、LCA、CD21、HMB45均阴性。诊断Langerhans细胞肉瘤。患者经放疗联合化疗，疗效不明显。于诊断后一年死亡。A 41-year-old male patient presented with masses at bilateral inguina and left waist for more than six months. The lesions were soft at the beginning, then gradually grew large, hardened and painful. Two weeks prior to the presentation, the masses ruptured. Routine blood test was normal and serum glucose level was a little higher. Since the tumors poorly responded to treatment with antibiotics, surgical excision was carried out. The masses were found to be located in subcutaneous tissue with a well-defined boundary, but synechia was severe with focal necrosis in the tumors. Pathologically, there were a large quan- tity of tumor cells diffusely arranged into patches in dermis and subcutaneous tissue; most of the cells were large with plenty eosinophilic cytoplasm as well as large, irregular nuclei lobulated or with concave marks. Some cells had longitudinal nuclear grooves and marked nucleoli. Also, numerous multinucleated giant cells were observed. Immunohistochemistry revealed that tumor cells were positive for S-100 protein, CDla and vimentin, weakly positive for lysozyme, partly positive for CD68, but negative for CD20, CD3, CD30, CKpan, actin, EMA, AFP, LCA, CD21 and HMB45. A diagnosis of Langerhans cell sarcoma was made. There was no obvious response to radiotherapy combined with chemotherapy, and the patient died within one year after the confirmation of diagnosis.

关 键 词：郎格尔汉斯细胞肉瘤 病理学 预后 

分 类 号：R739.5[医药卫生—肿瘤]