小肠系膜滤泡树突状细胞肉瘤伴盆腔转移的临床病理特征和免疫表型分析

Clinicopathological Features and Immunophenotypic Analysis on Follicular Dendritic Cell Sarcoma in Mesentery of Small Intestine with Pelvic Metastases

目的:探讨滤泡树突状细胞肉瘤的临床病理特征、免疫表型及鉴别诊断,提高对该肿瘤的认识,避免误诊。方法:对1例小肠系膜滤泡树突状细胞肉瘤行HE染色、免疫组化EnVison法染色观察其特点,并结合相关文献复习。结果:滤泡树突状细胞肉瘤排列成束状、漩涡状或弥漫成片,肿瘤细胞呈梭形或卵圆形,细胞界限不清,胞质嗜酸性,核卵圆形或长梭形,染色质空泡状,可见双核或多核瘤巨细胞,核分裂象约3个/10个高倍镜视野,间质见较多淋巴细胞及组织细胞聚集。免疫组化显示肿瘤细胞CD21、CD23、CD35、D2-40、趋化因子配体13(CXCL-13)、表皮生长因子受体(EGFR)、生长抑素受体2a(SSTR2a)、Vimentin阳性。分子病理结果为EBER-CISH(-),病理诊断为小肠系膜滤泡树突状细胞肉瘤。结论:滤泡树突状细胞肉瘤是一种罕见的低至中度恶性肿瘤,诊断依赖于组织病理学和免疫组化染色,该瘤治疗首选手术切除,预后尚不确定。

Objective:To investigate the clinicopathological characteristics,immunophenotype and differential diagnosis of the follicular dendritic cell sarcoma,thus improve the understanding of this tumor to avoid misdiagnosis.Methods:A case of follicular dendritic cell sarcoma in mesentery of small intestine with pelvic metastases was stained with HE staining and EnVison immunohistochemical staining,to observe its characteristics and review the related literatures.Results:The follicular dendritic cell sarcoma was arranged in fascicular,whorled and diffuse.Tumor cells were spindle or ovoid with unclear boundaries.The cytoplasm was eosinophilic,and the nuclear was oval or spindle with chromatin vacuolated.There were binucleate or multinucleate giant cells and three mitotic images can be seen in every ten high power fields.There are many lymphocytes and histamine aggregates in the stroma.Immunohistochemical results suggested tumor cells were positive for CD21,CD23,CD35,D2-40,CXCL-13,EGFR,SSTR2 a and Vimentin.Molecular pathology indicated it was negative to EBER-CISH.Pathological diagnosis was small intestinal follicular dendritic cell sarcoma.Conclusion:Follicular dendritic cell sarcoma is a rare,and low to moderate malignancy,which can be diagnosed by histopathology and immunohistochemical staining.The surgical excision is the preferred treatment,while the prognosis of this tumor is uncertain.