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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,1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。
Objective: To investigate the clinical manifestations, pathological features, treatment and prognosis of dendritic cell tumors. Methods: Among 4 patients with nasal and pharyngeal dendritic cell tumor, 2 cases of follicular dendritic cell sarcoma (FDCS), 1 case of Langerhans histiocytosis (LCH), 1 case of Langerhans cell sarcoma (LCS) 1 case. One of 2 FDCS patients underwent radiotherapy and chemotherapy combined with radiotherapy and chemotherapy. One patient underwent radiotherapy and chemotherapy. One patient with LCH and one patient with LCS underwent surgery. Results: In 2 patients with FDCS, one patient achieved complete remission after 4 cycles of CHOP and 50 Gy concurrent radiotherapy, and 1 patient had survival after 7 months of follow-up. One patient with LCH was followed up Two years later, no tumor recurrence and metastasis; 1 case of LCS children, no chemotherapy after surgery, followed up for 10 months after the death. CONCLUSION: Dendritic cell tumors are rare and clinically misdiagnosed. Pathological examination, histopathology, immunohistochemistry and electron microscopy are common. FDCS, LCH and LCS have unique pathological features, immunophenotypes, diagnosis and prognosis.