炎性假瘤样滤泡树突细胞肉瘤6例临床病理特征及预后分析  

Inflammatory pseudotumor-like follicular dendritic cell sarcoma: clinicopathological features and prognosis study of 6 cases

作　　者：向春香[1,2] 王建军[1] 吴楠[1] 余波[1] 夏春[1] 何燕[1] 马捷[1] 石群立[1] 

机构地区：[1]南京军区南京总医院病理科,南京210002 [2]武汉市中心医院病理科,430014

出　　处：《临床与实验病理学杂志》2014年第7期751-754,共4页Chinese Journal of Clinical and Experimental Pathology

摘　　要：目的 探讨炎性假瘤样滤泡树突细胞肉瘤（inflammatory pseudotumor-like follicular dendrtic cell sarcoma,IPT-likeFDCS）的临床病理学特征。方法 回顾性分析6例IPT-like FDCS的临床病理资料、免疫表型及EBER原位杂交结果,并追踪患者预后。结果 6例IPT-likeFDCS中女性4例,男性2例,年龄40～78岁,平均62.3岁。临床表现无特异性,4例患者为体检时发现,1例患者有腹胀、消瘦症状,1例患者有腰背部疼痛症状。3例患者肿瘤位于脾脏,2例位于肝脏,1例位于腹膜后。肿瘤直径3.5～14cm（平均7.8cm）,肿瘤通常与周围组织分界较清晰。肿瘤组织由圆形、卵圆形及梭形细胞组成,细胞异型性不明显,呈编织状、漩涡状或席纹状排列,其间见多量炎细胞混杂。免疫表型：肿瘤细胞表达CD21/35、vimentin及Clusterin。EBER原位杂交显示瘤细胞散在阳性。所有患者均经手术完整切除肿瘤,术后随访3～48个月,6例中5例患者无瘤生存,1例患者随访4年后（无复发和转移）失访。结论 IPT-like FDCS是一种好发于女性的少见低度恶性肿瘤,最常见的发病部位是脾脏和肝脏,与EB病毒感染有关,其生物学行为相对惰性,手术完整切除肿瘤是最佳治疗方式。Purpose To explore the clinicopathological features of inflammatory pseudotumor-like follicular dendrtic cell sarcoma （ IPT-like FDCS）. Methods Six patients diagnosed with IPT-like FDCS were retrospectively observed and analyzed by means of clinicopathological data, immunohistochemical staining and in situ hybridization for Epstein-Barr virus （EBV）-encoded RNA （EBER）. Results Among the 6 cases diagnosed as IPT-like FDCS, four cases were female, two cases were male, aged from 40 to 78 years （mean 62.3 years）. The clinical symptoms were non-specific, which four cases were documented by physical examination, one had abdominal distension and emaciation, and one had low back pain with the tumor in the retroperitoneal. The neoplasms, with a mean size 7.8 cm （range, 3.5 to 14 cm） ,＇occurred in the spleen （three cases）, liver （two cases）and retroperitoneal （one case）, which were usually slow-growing. The neoplasms usually had well-defined borders, composed of round, oval and spindle cells. The optical management of dysplasia were not obvious and they were arranged in whorls and a spiral or storiform growth pattern, mixed with inflam- matory cells. Immunohistochemically, tumor ceils were positive for CD21/35, vimentin and Clusterin protein, with EBER in situ hybridization scattered positive. All cases went complete surgical excision of tumors and were followed up for 3 - 48 months, with 5 patients disease-free and 1 patient lost follow-up （disease-unknown）. Conclusions IPT-like FDCS is a rare low-grade malignant tumor associated with EBV infection with a relatively indolent biological behavior, which is female predominated and mainly occurs in spleen and liver, and complete surgical excision may be the optimal approach to be performed.

关 键 词：炎性假瘤 滤泡树突细胞 肉瘤 EBER 

分 类 号：R735[医药卫生—肿瘤]