Su Zheng, Liu Gaojie, Liu Jianping, Fang Tingfeng, Zeng Yunjie, Zhang Huayao, Yang Shanglin, Wang Yang, Zhang Junmin, Wei Jinxing, Li Yingru, Guo Youfeng
Department of Comprehensive, Sun Yat-Sen Memorial Hospital, Sun Yat-Sen University Guangzhou 510120, China.
Department of Gastrointestinal Surgery, Sun Yat-Sen Memorial Hospital, Sun Yat-Sen University Guangzhou 510120, China.
Int J Clin Exp Pathol. 2015 Oct 1;8(10):11983-94. eCollection 2015.
Follicular dendritic cell sarcoma (FDCS) is a rare tumor associated with paraneoplastic pemphigus. It is Blame drenchs auxiliary cell tumor which is derived from the peripheral lymphoid tissues. Throughout the world, several patients of paraneoplastic pemphigus associated follicular dendritic cell sarcoma were reported in the literature, but mostly originated from the neck lymph nodes, and extranodal origin of follicular dendritic sarcoma was rarely reported. Also, so far we have found that the malignant degree of all patients diagnosed with malignant tumors have been reported were low and after combined treatment of surgery, radiotherapy and chemotherapy, most of the prognosis was good. However, here we present a patient of paraneoplastic pemphigus associated with follicular dendritic cell sarcoma origined from outside of the lymph nodes and had high tumor malignant degree for its unclear cell boundaries, obvious atypia and mitoses and the patient's state became progressively deteriorate after operation.
滤泡性树突状细胞肉瘤(FDCS)是一种与副肿瘤性天疱疮相关的罕见肿瘤。它是源自外周淋巴组织的一种辅助细胞肿瘤。在全球范围内,文献报道了数例副肿瘤性天疱疮相关的滤泡性树突状细胞肉瘤患者,但大多起源于颈部淋巴结,而结外起源的滤泡性树突状肉瘤鲜有报道。此外,迄今为止我们发现,所有已报道的被诊断为恶性肿瘤的患者恶性程度均较低,经过手术、放疗和化疗的联合治疗后,大多数患者预后良好。然而,在此我们报告一例起源于淋巴结外的副肿瘤性天疱疮相关滤泡性树突状细胞肉瘤患者,该肿瘤细胞边界不清、异型性明显且有核分裂象,恶性程度高,患者术后病情逐渐恶化。