Abstract:

Objective  to report one case of follicular dendritic cell sarcoma complicated with paraneoplastic pemphigus clinical manifestations and clinical treatment, so as to improve the understanding of the diseases clinical manifestations, to avoid misdiagnosis and missed diagnosis. Methods The clinical data including the manifestation and surgical treatment in one case of follicular dendritic cell sarcoma complicated with paraneoplastic pemphigus was introduced and analyzed, and the present related documents was review. Results Pathological findings showed the tumor arised from follicular dendritic cell sarcoma outside the lymph node, and the tumor cell boundary was unclear and had obvious atypia and mitosis. Because of the high malignancy of the tumor, the patient s condition deteriorated gradually. Conclusion For localized lesions, surgical resection is the preferred treatment for Follicular dendritic cell sarcoma complicated with paraneoplastic pemphigus. But surgery alone has a high local recurrence rate, consolidation radiotherapy can reduce the local recurrence rate of patients with surgery and extend its disease-free survival. For patients with systemic diffuse FDCS (multiple lymphadenopathy), there is a huge mass or surgery can not cure patients, the need for effective combination chemotherapy.

Key words: follicular dendritic cells, paraneoplastic, pemphigus

摘要：

目的 报告1例滤泡树突状细胞肉瘤并发副肿瘤性天疱疮的临床表现及临床治疗，从而提高对该疾病多样临床表现的认识，避免误诊、漏诊的发生并完善相关诊治。方法 分析1例滤泡树突状细胞肉瘤并发副肿瘤性天疱疮的临床表现及临床治疗并查阅国内外文献资料。结果 此例是来自淋巴结外部的滤泡性树突状细胞肉瘤，因其细胞边界不清，明显的异型和有丝分裂而具有较高的肿瘤恶性程度，手术后患者的状态逐渐恶化。结论 对于局限性病变，手术切除是首选治疗方式。但单纯手术具有较高的局部复发率，巩固性放疗可降低手术患者的局部复发率，并延长其无病生存期。对全身播散性FDCS（多处淋巴结肿大）、有巨大肿块或手术未能根治的患者，需要进行有效的联合化疗。

关键词: 天疱疮 , 滤泡树突状细胞, 副肿瘤