鼻及咽部树突细胞肿瘤4例临床分析并文献复习

目的：探讨树突细胞肿瘤的临床表现、病理特点、治疗和预后。方法：4例鼻及咽部树突细胞肿瘤患者中，滤泡状树突细胞肉瘤（FDCS）2例，朗格汉斯组织细胞增多症（LCH）1例，朗格汉斯细胞肉瘤（LCS）1例。2例FDCS中有1例行综合治疗（手术加放化疗），1例仅行放化疗；1例LCH及1例LCS患儿均行手术治疗。结果：2例FDCS患者，l例术后行4个疗程CHOP方案及同步放疗（50Gy）后达完全缓解，1例仅行放化疗者随访7个月仍带瘤生存；1例LCH患儿随访2年余，肿瘤无复发及转移；1例LCS患儿，术后未行放化疗，随访10个月后死亡。结论：树突细胞肿瘤罕见，临床易误诊，确诊需病理组织形态、免疫组织化学及电镜相结合，FDCS、LCH及LCS具有独特的病理表现、免疫表型、诊治及预后。

Diagnosis and management of nasal and pharyngeal dendritic cell tumor-case report and literature review

Objective：To investigate the clinical manifestation , pathological characteristics, treatment and prognosis of dendritic cell tumor. Method.. Four cases of nasal and pharyngeal dendritic cell tumor were described, including two ca- ses of follicular dendritic cell sarcoma （FDCS）, one case of Langerhans cell histiocytosis （LCH） and one case of Langer- hans cell sarcoma （LCS）. One of the patients with FDCS received multimodality therapy （surgery combined with chemo- therapy）, and the other patient only received chemotherapy and radiotherapy. The patients with LCH or LSC were treated by surgery. Result：Of the two FDCS patients, one achieved complete remission after treatment by surgery combined with four cycles of CHOP chemotherapy regimen and concurrent radiotherapy（50 Gy）, and the other who only received chemo- therapy and radiotherapy survived with tumor for more than seven months of follow up. The patient of LCH was followed up for more than 2 years after surgery without recurrence or metastasis. The patient of LCS did not undergo radiotherapy or chemotherapy after surgery and died after 10 months of follow up. Conclusion： Dendritic cell tumor is a group of very rare tumor and can be easily misdiagnosed in clinic, the confirmed diagnosis of which relies on histopathological features, immunohistochemistry combined with electron microscopy. FDCS, LCH and LCS have different pathological features, immunophenotypes and prognosis.