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肝血管周上皮样细胞肿瘤(PEComa):动态CT、MRI、超声及病理特征——7例分析并文献复习

Hepatic perivascular epithelioid cell tumor (PEComa): dynamic CT, MRI, ultrasonography, and pathologic features--analysis of 7 cases and review of the literature.

作者信息

Tan Yan, Xiao En-hua

机构信息

Department of Radiology, Second Xiangya Hospital, Central South University, Changsha, 410011, Hunan Province, China.

出版信息

Abdom Imaging. 2012 Oct;37(5):781-7. doi: 10.1007/s00261-012-9850-1.

Abstract

OBJECTIVE

To evaluate the dynamic CT, MRI, ultrasonography, and pathologic features of hepatic perivascular epithelioid cell tumor (PEComa), improving the understanding and diagnosis of the tumor.

METHODS

A retrospective analysis of CT, MRI, ultrasonography, and pathologic features of 7 hepatic PEComas diagnosed by pathology during 1st January 2005 to 1st September 2011 in our hospital.

RESULTS

The performance of dynamic CT, MRI, and ultrasonography revealed that lesions were regular masses with well-defined borders, the maximum diameters were 2.5-8.5 cm (mean = 4 cm), density was homogeneous, contrast-enhanced CT and MRI showed the lesions were significantly and heterogeneously enhanced on arterial phase, less enhanced on portal venous phase, and slightly hypodense on delayed phase. One patient had multiple hepatic lesions and had delayed enhancement. There were no backgrounds of hepatitis and cirrhosis, enlarged lymph nodes, or distant metastases. Pathology showed the gross appearance of the tumor was smooth. Tumor cells were round or polygonal, with clear boundaries and clear membranes, and had abundant translucent cytoplasm. Nuclei were round, with medium size. Tumor cells were epithelial-like cells and arranged in dense sheets. Immunohistochemistry showed that most of them were positive in HMB45 and MelanA, S-100, SMA, while negative in CgA, Syn, CK, CD117, CD10, and CD34.

CONCLUSIONS

Dynamic CT, MRI, ultrasonography, and pathology of PEComa had some characteristics of benign tumor's performance. Enhanced scan showed PEComa quickly enhanced on arterial phase and enhanced less on portal venous phase. Knowing these characteristics could help to improve the understanding and diagnosis of hepatic PEComa.

摘要

目的

评估肝脏血管周上皮样细胞瘤(PEComa)的动态CT、MRI、超声及病理特征,提高对该肿瘤的认识及诊断水平。

方法

回顾性分析2005年1月1日至2011年9月1日我院经病理确诊的7例肝脏PEComa的CT、MRI、超声及病理特征。

结果

动态CT、MRI及超声表现为边界清晰的规则肿块,最大径2.5~8.5 cm(平均4 cm),密度均匀,CT及MRI增强扫描显示病灶动脉期明显不均匀强化,门静脉期强化程度减低,延迟期呈轻度低密度。1例患者肝脏有多发病灶且有延迟强化。无肝炎、肝硬化背景,无肿大淋巴结及远处转移。病理显示肿瘤大体形态光滑。肿瘤细胞呈圆形或多边形,边界清晰,胞膜清楚,胞质丰富、透亮。细胞核圆形,中等大小。肿瘤细胞为上皮样细胞,呈致密片状排列。免疫组化显示多数病例HMB45、MelanA、S-100、SMA阳性,而CgA、Syn、CK、CD117、CD10及CD34阴性。

结论

PEComa的动态CT、MRI、超声及病理表现具有一些良性肿瘤的特征。增强扫描显示PEComa动脉期快速强化,门静脉期强化程度减低。了解这些特征有助于提高对肝脏PEComa的认识及诊断水平。

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