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 IPTlike 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 hyperenhancement 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
【中图分类号】R333.4
【文献标识码】A
【DOI】10.3969/j.issn.1009-3257.2017.04.016
前言
肝炎性假瘤样滤泡树突细胞肉瘤(inflammatory pseudotumor like follicular dendritic cell sarcoma,IPT-like FDCS)是一种十分罕见的树突细胞 来源的恶性肿瘤,具备部分炎性假瘤的组织学特征。 目前国内外报道较少,本文报道一例肝炎性假瘤样滤 泡树突细胞肉瘤,结合相关文献对其临床表现、影像 学特点、诊断、治疗及预后进行分析。
罕少疾病杂志
第24卷, 第 4 期
2019年11月
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