滤泡树突状细胞肉瘤的临床病理观察  

Follicular dendritic cell sarcoma： a clinicopathologic study of 8 cases

作　　者：张正祥[1] 程静[1] 石群立[1] 马捷[1] 周晓军[1] 周航波[1] 马恒辉[1] 

机构地区：[1]南京大学医学院临床学院南京军区南京总医院病理科,210002

出　　处：《中华病理学杂志》2008年第6期395-399,共5页Chinese Journal of Pathology

摘　　要：目的探讨和分析滤泡树突状细胞肉瘤（FDCS）的临床病理特点及鉴别诊断。方法应用组织学、免疫组织化学（EnVision法）标记及EBER原位杂交，对8例FDCS进行临床和组织病理学分析，并复习相关文献。结果8例FDCS中男性5例，女性3例，平均年龄50岁。发生部位淋巴结4例，扁桃体、鼻咽部、肝、脾各1例。组织学：瘤组织呈席纹状、束状、弥漫性、旋涡状或结节状，肿瘤细胞呈合体样，境界不清，胞质较丰富，均质嗜伊红或细颗粒状，核呈卵圆形、短梭形或圆形，染色质稀疏或呈空泡状、点彩状，核仁明显，核分裂象多少不等，肿瘤细胞间见有散在淋巴细胞混杂。间质内可见假血管腔及血管周围淋巴鞘现象。其中肝脏1例以大量小淋巴细胞弥漫分布为背景，梭形或卵圆形的瘤细胞散在分布其中，瘤细胞核染色质细腻，部分区域细胞有轻度异形，核不规则、空泡状，有核仁。免疫组织化学瘤细胞均表达CD21、CD35、clusterin，部分表达CD68、上皮细胞膜抗原、S-100及内皮生长因子受体，Ki-67不同程度表达。EBER两例表达。结论FDCS是一种非常少见的恶性肿瘤，易复发和转移，明确诊断需要结合病理形态学和免疫表型。Objective To investigate clinicopathologic features, immunophenotypes and differential diagnoses of follicular dendritic cell sarcoma/tumor （FDCS）. Methods Eight cases of FDCS were studied using histological and immunohistochemical examinations and EBER in situ hybridization, with a review of the related literatures. Results There were 5 male and 3 female patients with a median age of 50 years. The sites of involvement included lymph node （ 4 cases）, tonsil, nasopharynx, liver, and spleen （ 1 case each, respectively）. The predominant microscopic features histologically included storiform, fascicular, diffuse, whorled and nodular in patterns. The neoplastic cells, dispersed by the infiltrated small lymphoeytes, were characterized by abundant eosinophilie or fine granular cytoplasm with indistinct cell borders, and syncytial in appearance. The nuclei of the tumors were ovoid, round to spindled in shape with vesicular or stippled chromatin and small distinct nucleoli. Mitotic figures varied among cases. Pseudovascular spaces and perivascular cuffing were observed in some cases. One case of FDCS involving lesion in liver showed a background of abundant lymphocytes mixing with dispersed spindle or ovoid neoplastic cells having delicate chromatin, mild nuclear atypia, irregular/vesicular nuclei and distinct nucleoli. The neoplastic cells were positive for CD21, CD35, clusterin, and weakly positive for CD68, EMA, S-100 and EGFR. Ki-67 stain showed a variable expression among cases. EBER was positive in 2 cases. Conclusions FDCS is a rare malignant tumor with a tendency to relapse and metastasis. Combined morphological and immunophenotypical analysis is necessary to reach a correct diagnosis.

关 键 词：树突细胞 滤泡 肉瘤 原位杂交 

分 类 号：R686[医药卫生—骨科学]