肝脏滤泡树突状细胞肉瘤的临床病理分析

Clinical and pathologic characteristics of hepatic follicular dendritic cell sarcoma

目的探讨肝脏滤泡树突状细胞肉瘤临床病理特征和免疫表型,观察其生物学行为及临床治疗效果。方法对1例肝脏滤泡树突状细胞肉瘤进行光镜观察和免疫组织化学检测,观察其生物学行为及临床治疗效果。结果光镜检查:梭形,卵圆形的肿瘤细胞排列成束状,巢片状;肿瘤细胞间见较多的淋巴细胞,肿瘤内见凝固性坏死。肿瘤细胞核呈空泡状,核仁明显,细胞浆丰富,细胞边界不清,呈合体状。免疫表型:瘤细胞弥漫表达CD21、CD68、灶状表达CD35和S-100,而D2-40、CD1a、CD31、CD34、EBV、Hepa-1和AFP均阴性。肿瘤的生物学行为及临床治疗过程:肿瘤首先表现为肝脏肿块,经过手术及辅助治疗后,肿瘤反复复发,转移。结论肝脏淋巴结外滤泡树突状细胞肉瘤是一种罕见的免疫辅助细胞中度恶性肿瘤,术后容易复发甚至转移,应予以完整切除,并辅以合适的化疗和(或)放疗。

[Objective]To study the clinicopathologic features, immunophenotype, biological behaviour and management of hepatic follicular dendritic cell sarcoma（FDCS）.[Method] One case of hepatic FDCS was evaluated by light microscopy and immunohistochemistry, meanwhile biology behavior and response to treatment was observed. [Result]The tumor was composed of sheets, and fascicles of oval-to-spindle cells intermingled with the infiltrates of small lymphocytes, areas of coagulative necrosis were found. The cells had vesicular nuclei, distinct nucleoli, indistinct cell borders and syncytial cytoplasm. Immunohistochemically, the tumour cells were positive for CD21 and CD68, focal positive for CD35, S-100 but negative for D2-40, CD1a, CD31, CD34, EBV, Hepa-1, AFP. biological behaviour and clinical therapy process of the tumor goes as follow, it present with a hepatic mass at first, underwent a mass resection followed with adjunctive therapy, the tumor relaps and metastasis repeatly. [Conclusion] Extranodal follicular dendritic cell sarcoma in liver is an extremely rare entity of the accessory immune cells which should be viewed as a tumor of intermediate malignancy and treated by complete excision with adjuvant chemotherapy and （or） radiotherapy.