原发性肝脏纤维肉瘤CT检查影像学特征

CT features of primary hepatic fibrosarcoma

目的探讨原发性肝脏纤维肉瘤(PHF)CT检查影像学特征。方法采用回顾性描述性研究方法。收集2010年1月至2019年12月国内2家医疗中心收治的8例(乐清市人民医院3例和温州市人民医院5例)PHF病人的临床病理资料;男5例, 女3例;年龄为50岁(39~60岁)。病人术前均行CT平扫和增强扫描检查, 根据病人意愿和个体情况选择治疗方式。术后将肿瘤标本进行病理学检查和免疫组织化学染色检测。观察指标:(1)CT检查影像学特征。(2)治疗及病理学检查情况。(3)随访情况。采用门诊或住院方式进行随访, 了解病人生存和肿瘤复发转移情况。随访时间截至2020年12月。偏态分布的计量资料以M(范围)表示, 计数资料以绝对数表示。结果 (1)CT检查影像学特征:8例病人术前均行CT检查, 诊断为恶性肿瘤3例, 肝内占位性病变3例, 肝内胆管细胞癌2例。①肿瘤部位和大体观:8例病人肿瘤均为单发, 肿瘤位于肝右叶6例, 肝左叶2例;肿瘤呈椭圆形5例, 圆形3例;肿瘤长径为9.3 cm(4.0~15.0 cm), 其中长径≤5.0 cm 1例, 5.1~10. 0 cm 4例, >10.0 cm 3例。②CT平扫检查结果显示:8例病人肿瘤均呈低密度, CT值为40 HU(29~43 HU), 其中密度不均匀6例, 密度均匀2例;肿瘤呈膨胀性生长界限清晰6例, 肿瘤呈浸润性生长界限不清晰2例;瘤内坏死、囊变5例, 瘤内条片状出血灶2例, 瘤内小片状钙化1例;肝门区淋巴结转移1例。③CT增强扫描检查结果显示:8例病人CT增强扫描检查动脉期示肿瘤呈不均匀强化, 门静脉期示肿瘤密度不均匀, 延迟期示肿瘤强化均缓慢退出, 密度低于肝实质。(2)治疗及病理学检查情况:①治疗情况包括8例病人均行手术切除治疗, 肝内和肝门区未见转移。②肿瘤病理学检查结果显示:8例病人中, 2例肿瘤分化为Ⅰ级(低度恶性), 5例肿瘤分化为Ⅱ级和Ⅲ级(中度恶性), 1例肿瘤分化为Ⅳ级(高度恶性)。③免疫组织化学染色检测结果显示:8例病人波形蛋白、内皮细胞10、p53均为阳性, 平滑肌肌动蛋白、角蛋白19、上皮膜抗原、内皮细胞34均为阴性;8例均确诊为PHF。(3)随访情况:8例病人术后均获得随访, 随访时间为7~128个月, 中位随访时间为53个月。8例病人总生存时间为15~122个月, 中位总生存时间为45个月;其中1例于术后17个月因肿瘤复发和远处转移死亡, 7例生存时间>28个月。结论 PHF CT平扫检查示不均匀低密度肿块, 增强扫描检查动脉期示肿瘤环形或周边结节状不均匀强化, 瘤内条状强化血管, 门静脉期示瘤内小片状或网格样持续强化, 延迟期示瘤内强化缓慢退出。

Objective To investigate the computed tomography(CT)features of primary hepatic fibrosarcoma(PHF).Methods The retrospective and descriptive study was conducted.The clinicopathological data of 8 patients with PHF who were admitted to 2 medical centers from.January 2010 to December 2019,including 3 cases of Yueqing People's Hospital of Zhejiang and 5 cases of Wenzhou People's Hospital of Zhejiang were collected.There were 5 males and 3 females,aged 50 years(range,39-60 years).All patients underwent abdominal CT plain and enhanced examination,and surgical procedures were selected based on patients'will and individual situations.Surgical tumor specimens were given pathological examination and immunohistochemical staining.Observation indicators:(1)imaging features of CT examination;(2)treatment and pathological examinations;(3)follow-up.Follow-up was performed by outpatient examination or hospitalization to detect survival of patients and tumor recurrence up to December 2020.Measurement data with skewed distribution were represented as M(range).Count data were described as absolute numbers.Results(1)Imaging features of CT examination:8 patients received preoperative CT examinations,3 cases of which were diagnosed as malignancies,3 cases were diagnosed as hepatic space-occupying lesions,2 cases were diagnosed as intrahepatic cholangiocarcinoma.①Tumor location and general status:8 patients had single tumor,of which 6 cases had tumor located in the right lobe of the liver and 2 cases had tumor located in the left lobe of the liver.The tumor was in oval shape in 5 patients and in circular in 3 patients.The maximum diameter of tumor in 8 patients was 9.3 cm(range,4.0-15.0 cm).There were 1 case with tumor maximum diameter≤5.0 cm,4 cases with tumor maximum diameter as 5.1-10.0 cm,3 cases with tumor maximum diameter>10.0 cm.②CT plain scan showed uniform low density in 2 cases and uneven low density in 6 cases and the CT value of 8 cases was 40 HU(range,29-43 HU).The tumor showed expansive growth with clear boundary in 6 cases,and invasive growth with unclear boundary in 2 cases.Five cases had intratumoral necrosis and cystic transformation,2 cases had intratumoral strip hemorrhage,and 1 case of intratumoral calcification.There was 1 case with hilar lymph node metastasis.③Enhanced CT scan showed 8 cases with uneven enhancement in arterial phase,8 cases with uneven tumor density in portal vein phase,8 cases with intratumoral enhancement slowly withdrew in delayed phase and the density lower than liver parenchyma.(2)Treatment and pathological examinations:①8 patients under-went surgical resection,without intrahepatic or hilar metastasis.②The results of postoperative pathological examination showed 2 cases with grade Ⅰ tumor differentiation(low potential malignancy),5 cases with grade Ⅱ and Ⅱ tumor differentiation(moderate potential malignancy),1 case with grade IV tumor differentiation(high potential malignancy).③Immuno-histochemical staining showed that 8 cases were positive for vimentin,CD10,p53 and negative for smooth muscle actin,keratin19,epithelial membrane antigen,endotheliocyte 34.All 8 patients were diagnosed as PHF.(3)Follow-up:all 8 patients were followed up for 7 to 128 months,with a median follow-up time of 53 months.The overall survival time of 8 patients was 15 to 122 months,and the median overall survival time was 45 months.One patient died of tumor recurrence and distant metastasis at 17 months after surgery,and the survival time of 7 patients was>28 months.Conclusions CT plain scan of PHF showed uneven low-density masses.After enhancement,the arterial phase shows the tumor ring or peripheral nodular uneven enhancement,the intratumoral strip-shaped enhanced blood vessels.The portal vein phase shows the tumor-like small patches or grid-like continuous enhancement.The delayed phase is characterized by the slow withdrawal of intratumoral enhancement.