肝脏炎性假瘤样滤泡树突细胞肉瘤一例及文献复习  

A Inflammatory Pseudotumor-like Follicular Dendritic Cell Sarcoma: A Case Report and Literature Review

作　　者：陈燕[1] 孙德胜[1] 林晓娜 黎永滨[1] 蔡华丽[1] CHEN Yan SUN De-sheng LIN Xiao-na et al(Department of Ultrasound, Beijing University Shen Zhen Hospital, Shenzhen 518035, Guangdong Province, China)

机构地区：[1]北京大学深圳医院超声科,广东深圳518035

出　　处：《罕少疾病杂志》2017年第4期35-37,共3页Journal of Rare and Uncommon Diseases

摘　　要：目的探讨肝脏炎性假瘤样滤泡树突细胞肉瘤的临床诊断及影像学特点。方法回顾性分析一例肝炎性假瘤样滤泡树突细胞肉瘤患者的临床资料及影像学检查,并复习有关文献。结果肝炎性假瘤样滤泡树突细胞肉瘤是一种极为罕见的低度恶性肿瘤,临床表现为不明原因发热、消瘦,血清学肿瘤标志物阴性。超声表现为肝内巨大实性占位,边界清晰。彩色多普勒显示肿块周边血流信号,内部血流信号稀少。CT增强动脉期病灶不均匀性强化,门脉期、延迟期消退不明显,中央多见液化坏死无增强区。结论肝脏炎性假瘤样滤泡树突细胞肉瘤影像学具有一定的特点,但特异性不明显。主要依靠病理组织学及免疫组化确诊,手术切除肿瘤是有效的治疗手段。Objective To investigate the clinical diagnosis and imaging characteristic of inflammatory pseudotumor like follicular dendritic cell sarcoma （IPT-like FDCS）, so as to strength the cognition for IPT-like FDCS. Methods The clinical data and imaging information of a case of IPT-like FDCS were retrospectively investigated. Results IPT-like FDCS was an uncommon tumor which with low potential of malignancy. The most common clinical manifestations of IPT- like FDCS were fever with unknown cause, weight loss, while usually the serological tumor markers were negative. On ultrasonography IPT-like FDCS appeared as a huge entity Intrahepatic which was well defined. Color flow surrounding the entity can be observed on color Doppler imaging（CDFI）, while color flow may be scarce within the lesion. On contrast-enhanced computer tomography（CT） imaging, IPT-like FDCS appeared heterogeneous hyper- enhancement at the arterial phase, and the manifestation of ＂wash-out＂ was mild or even absent at the portal and delay phase. Central none-enhancement zone which may indicated necrosis or hemorrhage can usually be observed. Conclusion IPT-like FDCS had some characteristic imaging features, but the features were without significant specificity. The definite diagnosis depends on histopathological and immunohistochemical examination. Surgical resection of the tumor is the standard treatment.

关 键 词：炎性假瘤样滤泡树突细胞肉瘤 临床表现 影像学特点 病理 

分 类 号：R333.4[医药卫生—人体生理学]