关键词: 天疱疮 , 滤泡树突状细胞, 副肿瘤

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