原发性肝脏指状突树突细胞肉瘤临床病理特征  

Clinicopathological features of primary hepatic interdigitating dendritic cell sarcoma

作　　者：陈鸿远 董辉 俞花 朱玉瑶 余秋凤 钱尤雯 CHEN Hong-yuan;DONG Hui;YU Hua;ZHU Yu-yao;YU Qiu-feng;QIAN You-wen(Department of Pathology,Third Affiliated Hospital,Naval Medical University,Shanghai 200438,China;Department of Pathology,First Affiliated Hospital,Naval Medical University,Shanghai 200433,China;Student Brigade,School of Basic Medicine,Naval Medical University,Shanghai 200433,China)

机构地区：[1]海军军医大学第三附属医院病理科,上海200438 [2]海军军医大学第一附属医院病理科,上海200433 [3]海军军医大学基础医学院学员队,上海200433

出　　处：《临床与实验病理学杂志》2023年第2期169-173,共5页Chinese Journal of Clinical and Experimental Pathology

基　　金：上海市科委“科技创新行动计划”医学创新研究专项(22Y11908700)。

摘　　要：目的 探讨肝脏指状突树突细胞肉瘤(interdigitating dendritic cell sarcoma, IDCS)的临床病理学特征、免疫表型、分子改变、鉴别诊断及预后。方法 收集2例肝脏IDCS的临床资料,行HE、免疫组化染色及分子检测,分析其临床病理特征,并复习相关文献。结果 患者女性,年龄分别为39、67岁,肿瘤与周围肝组织分界清楚。镜下见肿瘤组织呈弥漫片、巢状分布,有凝固性坏死,对周围肝窦有侵犯,细胞卵圆形或多边形、细胞胞界不清、胞质嗜酸,核大空泡状,核膜及核仁明显,部分可见核沟,核分裂象易见,间质内有大量小淋巴细胞、浆细胞浸润,可见一定数量的嗜酸性粒细胞。免疫表型:vimentin、S-100、Fascin、CD68和CD163均阳性,CD4部分阳性,CD1a、Langerin(CD207)、CD21、CD23、CD35等均阴性,Ki-67增殖指数为10%~30%;MMR蛋白均阳性,微卫星检测均为微卫星稳定型。EBER原位杂交检测均为阴性;未检测到B细胞及T细胞克隆性重排。二代测序结果示:1例存在ERBB2、IDH1、FGFR3和KRAS基因错义突变,1例存在ERBB2、BRAF V600E和CREBBP基因错义突变。结论 原发性肝脏IDCS是一种极为罕见的组织细胞/树突细胞肿瘤,恶性度高,确诊主要依靠病理形态学及免疫组化检测,需与组织细胞肉瘤、Langerhans细胞肉瘤、转移性黑色素瘤等进行鉴别。Purpose To investigate the clinicopathological features, immunophenotype, molecular changes, differential diagnosis and prognosis of interdigitating dendritic cell sarcoma(IDCS) of the liver. Methods The clinical data of 2 cases of hepatic IDCS were collected, and HE, immunohistochemical staining and molecular detection were performed. The clinicopathological features were analyzed, and the relevant literatures were reviewed. Results The patients were female, aged 39 and 67 years, and the boundaries between the tumor and the surrounding liver tissue were clear. Microscopically, the tumor tissues showed a diffuse sheet and nest-like histological growth pattern, with coagulative necrosis, invasion of the surrounding hepatic sinuses. The tumor cells were oval or polygonal cells with indistinctive cell boundaries, eosinophilic cytoplasm, large and vacuolar nuclei. The nuclear membranes, nucleoli, nuclear grooves and mitosis were obvious. A large number of small lymphocytes and plasma cells could be seen in the stroma, with a certain number of eosinophils. Immunohistochemically, the tumor cells were positive for vimentin, S-100, Fascin, CD68, and CD163. CD4 was partially positive within tumor. The tumor cells were negative for CD1a, Langerin(CD207), CD21, CD23, CD35, etc. Ki-67 proliferation index was 10%-30%. All MMR proteins were positive, and microsatellite detection showed that they were microsatellite stable. EBER in situ hybridization was negative. No gene clonal rearrangement of B cells or T cells was detected. The next generation sequencing results showed that one case had missense mutations in ERBB2, IDH1, FGFR3 and KRAS genes, and the other case had missense mutations in ERBB2, BRAF V600E and CREBBP genes. Conclusion Primary hepatic interdigitating dendritic cell sarcoma is an extremely rare histiocytic/dendritic cell tumor with high malignancy. The diagnosis mainly depended on the results of pathomorphology and immunohistochemistry, which should be differentiated from histiocytic sarcoma, Langerhans cell sa

关 键 词：肝脏肿瘤 指状突树突细胞肉瘤 分子遗传学特征 ERBB2突变 

分 类 号：R735.7[医药卫生—肿瘤]