滤泡树突细胞肉瘤5例并文献复习  

Follicular Dendritic Cell Sarcoma: A Report of 5 Cases and Literature Review

作　　者：陈鹭姗[1] 杨映红[1] 

机构地区：[1]福建医科大学附属协和医院病理科,福建福州350001

出　　处：《湖北民族学院学报（医学版）》2014年第4期24-27,30,F0003,共6页Journal of Hubei Minzu University(Medical Edition)

摘　　要：目的：探讨滤泡树突细胞肉瘤（ FDCS）的临床病理特征、免疫表型、诊断及鉴别诊断。方法对我院存档5例FDCS病例进行临床病理形态分析及免疫组化标记，并复习相关文献。结果5例FDCS分别发生在左锁骨上淋巴结、鼻腔、扁桃体、小肠、空肠系膜，发病年龄31～60岁，中位年龄45岁。3例表现为增大的肿块伴有局部不适，2例表现为腹部不适及腹痛。大体上肿瘤呈灰白色，质地中等。镜下肿瘤细胞呈梭形或卵圆形，排列成片状、束状、席纹状或漩涡状，间质中见呈簇状分布的小淋巴细胞，发生于鼻咽者可呈上皮样形态，具有明显多形性，核分裂像易见。5例病例中CD21阳性率100％，CD23阳性率60％，CD35阳性率80％，上皮样类型还可表达CK与EBER等。随访4例，随访9～44个月。2例经手术切除并辅以化疗后一般状况良好，1例行手术活检后未行治疗，1例于确诊28个月后死于全身转移。结论 FDCS是一种发生于淋巴结内外的少见病例。虽然诊断困难且组织形态容易与其他肿瘤混淆，但通过免疫组化方法可予以正确诊断。Objective To study the clinicopathologic features, immunophenotype, diagnosis and differential diagnosis of follicular dendritic cell sarcoma.Methods Five cases of follicular dendritic cell sarcoma were evaluated by histological and immunohistochemical staining, and the related literatures were reviewed.Results The masses were detected in left supraclavicular lymph node, nasal cavity, tonsil, the small intestine, and mesojejunum respectively.The age ofpa-tients ranged from 31 to 60 years （ median=45 years ） .Three cas es presents as an enlarging solid mass and local disorders.The other two cases presented with abdominal discomfort or pai n.Grossly, all of the five cases showed grey-white color andmedian texture.Histologically, the neoplastic cells were s pindle-shaped or ovoid and arranged mostly in solid sheets,fasci-cles, whorls or storiform pattern with intermixed sm all lymphocytes.The neoplastic cells of case 2 were epithelioid with significant cytological atypia, and the mitotic we re readily demon-strated.Immunohistochemical study showed that all cases were positive for CD21.There was focal expression of CD23（3/5, 60%） and CD35（4/5,80%）.The epithelioid cells were positive for CK and EBER.Of the four cases with follow-up information available （ duration from 9 months to 44 months） , two cases were live well after surgical resection with adjuvant chemo-therapy.One case was alive without any therapy after diagnosis.One case died of metasis at 28 months.Conclusion FDCS is a rare neoplasm, which can be observed in lymph nodes or ex-tranodes.It is seemingly difficult to diagnose and easily confused with other similar lesions.Im-munohistochemical studies are necessary for its diagnosis and differential diagnosis.

关 键 词：滤泡树突细胞肉瘤 免疫组化 病理学 

分 类 号：R730.2[医药卫生—肿瘤]