指突状树突状细胞肉瘤诊治探讨

目的：探讨树突状细胞肉瘤的临床特征、诊断、治疗和预后。方法：对1例指突状树突状细胞肉瘤进行临床病理特点分析,CHOP（环磷酰胺、表阿霉素、长春地辛、地塞米松）方案化疗并观察疗效,复习文献总结国内树突状细胞肉瘤诊治情况。结果：临床表现为全身淋巴结肿大,颈部呈巨大肿块,两肺弥漫性微小结节,伴盗汗、轻度贫血。病理结果示淋巴结结构破坏,瘤细胞成梭形,形成束状,交错状排列。免疫组化S-100（＋）、Vimentin（＋）、Fascin（＋）、Lyso（＋）、Ki-67（＋）,部分肿瘤细胞CD68（＋）。经CHOP方案化疗后达到部分缓解。Langerhans细胞肉瘤罕见,呈高度恶性表现,常累及多个器官,目前国内仅3例报道。指突状树突状细胞肉瘤国内共报道6例,年龄19～41岁（中位33岁）,颈部淋巴结好发,手术后无病生存时间2～15月（中位10月）,18月总生存率40%（2/5）。滤泡树突状细胞肉瘤18例,年龄16～63岁（中位37岁）,发病部位1/3以上在结外,无病生存时间5～72月（中位11.5月）,18月总生存率100%（6/6）。结论：树突状细胞肉瘤诊断依赖病理和免疫组化;治疗尚需规范,可采取手术、放疗、化疗相结合的方案。

A case report of interdigitating dendritic cell sarcoma and analysis of the diagnosis and treatment of dendritic cell sarcomas in China

Objective:To study the clinical and pathological features and diagnosis,treatment and prognosis of dendritic cell sarcomas.Methods:The clinical and pathological characteristics of one case with interdigitating dendritic cell sarcoma were investigated.A review of related literature was also done to see the status of diagnosis and treatment of dendritic cell sarcomas in China.Results:The case presented lymphadenopathy,especially in her neck,and diffuse small nodules in lung,accompanied with mild anemia and night sweating.Microscopically,the lymph node structure was destroyed.The tumor composed of spindle cells arranged in fascicles,and a storiform pattern.Immunohistochemically,the tumor cell stained positively for S-100,Fascin,Vimentin,Lyso( ),Ki-67 and partly CD68( ).The patient achieved part remission after one cycle CHOP(cyclophosphamide,epirubicin,vindesine,dexamethasone) treatment.Langerhans cell sarcoma was rare with a high malignant manifestation involved in multiple organs.There were only 3 cases reported in China.There were 6 cases with interdigitating dendritic cell sarcoma reported in China ranging 19-41 years old(median 33).The predilection site was neck lymph nodes.The disease-free survival time after surgery was 2-15 months(median 10 months).Eighteen months overall survival rate was 40%(2/5).There were 18 cases with follicular dendritic cell sarcoma reported in China ranging 16-63 years old(median 37).The sarcoma occurred at extranodal sites in more than one third of patients.The disease-free survival time after surgery was 5-72 months(median 11.5 months).Eighteen months overall survival rate was 100%(6/6).Conclusion:Dendritic cell sarcomas are rare.A correct diagnosis can be difficult to make and immunohistochemical are required for the diagnosis.The best treatment regimen should be further studied.Surgery resection,radiation and chemotherapy should be combined.