肝脏原发性恶性黑色素瘤15例临床及影像学特征分析

Clinical and imaging features of primary malignant melanoma of liver

目的总结肝脏原发性恶性黑色素瘤的临床、影像学特征,提高本病的诊断水平。方法以原发性肝脏恶性黑色素瘤为关键词检索万方数据库1980~2014年间相关文献,总结肝脏原发性恶性黑色素瘤的临床、影像学特征。结果 2000年以前我国无该病例报道,2000年以后报道15例。原发于肝脏的恶性黑色素瘤较为罕见,其临床表现及CT表现无明显特征。MRI表现为T1和T2弛豫时间缩短,T1WI呈高信号,T2WI呈低信号。肝穿刺病理学检查可见异型细胞呈巢状分布,分化较差,大量黑色素沉积。免疫组化结果显示HMB45(+)、S-100(+)、CK(-)。结论肝脏原发性恶性黑色素瘤临床缺乏特异性症状,主要表现为上腹部胀满、疼痛和肝功异常等。MRI是诊断恶性黑色素瘤的最佳影像学检查方法,表现为T1和T2弛豫时间缩短,T1WI呈高信号,T2WI呈低信号。

Objective To summarize the clinical and imaging features of primary malignant melanoma of liver in order to raise the diagnosis of this disease. Methods We took ' Primary liver malignant melanoma' as the keywords to search the literatures from 1980 to 2014 in the Wanfang database and summarized the clinical and imaging features of primary malignant melanoma of liver. Results There was no case reported in China before 2000,and 15 cases reported after 2000. Primary malignant melanoma is relatively rare in the liver. Its clinical manifestations and CT show no obvious characteristics. MRI performance has certain characteristics: T1 and T2 relaxation time shortens,T1 WI shows high signal and T2 WI shows low signal. Liver biopsy: there is nest-like distribution of heterocyst,poor differentiation and a large amount of melanin deposition.Immunohistochemical: HMB45( +),S-100( +) and CK(-). Conclusions The clinical features of primary malignant melanoma of liver lack of special clinical symptoms,and the main manifestations are upper abdominal distension,pain and abnormal liver function and other symptoms. MRI is the best imaging examination method in the diagnosis of malignant melanoma,which shows that T1 and T2 relaxation time is shortened,T1 WI has high signal and T2 WI has low signal.