探讨原发性肝脏黑色素瘤（PHM）的临床表现和诊疗经验。

检索PubMed、万方、中国知网、维普数据库2020年8月31日之前发表的PHM相关文献资料。中文检索词为"肝"、"黑色素瘤"；英文检索式为"（primary） and （hepatic or liver） and （melanoma）"。

共纳入30篇文献，包括31例PHM患者；男19例，女12例，男女比例为1.58∶1；成人30例，儿童1例；平均年龄（47±15）岁。多以腹痛或右上腹、肝区胀痛不适为主要症状。术前误诊率达74%（23/31）。12例上腹部MRI检查T1加权像高信号，T2加权像低信号，肿块影强化程度不一。病理学检查见肿瘤细胞呈圆形、多角形或梭形，胞质中见大量灰黑色颗粒，核圆形或卵圆形，核仁明显，嗜酸或弱嗜碱性，核分裂象易见；免疫组化检测HMB45、Melan-A、Vimentin阳性率分别为20/20、8/8、6/6。治疗方案包括手术、化疗、介入、免疫治疗等。18例获得随访，随访时间0~12个月，1年生存率为30%，中位生存时间为5个月。2例随访期间存活未复发，1例接受单纯手术治疗，另1例接受手术+术后IL-2治疗。

PHM是一类恶性程度极高、极易误诊、预后较差的罕见病例，临床无明显特异性表现，上腹部增强MRI检查及病理免疫组化检查有助于明确诊断。早期手术及术后IL-2治疗可能延长生存时间。

To investigate the clinical manifestations, diagnosis and treatment experience of primary hepatic melanoma (PHM).

Literature related to PHM published before August 31, 2020 was searched using Chinese search terms "liver" and "melanoma" and English terms "(primary) and (hepatic or liver) and (melanoma)" in PubMed, Wanfang data, CNKI and Chongqing VIP databases.

30 articles, 31 PHM patients were included. 19 cases were male and 12 female witha male-to-female ratio of 1.58∶1, and 30 cases were adults and 1 child. The average age was (47±15) years. The main symptoms were abdominal pain or swelling pain and discomfort in the right upper abdomen or hepatic region. The preoperative misdiagnosis rate was 74%(23/31). High-signal on T1WI and low-signal on T2WI were observed by the upper abdomen MRI scan in 12 cases, and the density of lesions were manifested with varying degree of enhancement. Pathological examination found that the tumor cells were in round, polygonal or spindle shape, with a large quantity of gray-black particles in the cytoplasm, round or oval nucleus, clear nucleoli, eosinophilic or weakly-basophilic nature and obvious caryokinesis. The positive rates of HMB45, Melan-A and Vimentin were 20/20, 8/8 and 6/6, respectively, reported by immunohistochemical staining. The therapeutic scheme included surgery, chemotherapy, interventional therapy and immunotherapy, etc. 18 cases were followed up. The 1-year survival rate was 30% and the median survival time was 5 months. 2 patients survived without recurrence during the follow-up, including 1 case receiving surgery alone, and the other receiving surgery combined with postoperative IL-2 therapy.

PHM is a rare disease, which is characterized with high-degree malignancy, high misdiagnosis rate, poor prognosis and without specific clinical manifestations. Contrast-enhanced MRI of the upper abdomen, pathological examination and immunohistochemical staining contribute to confirm the diagnosis. Early surgery combined with postoperative IL-2 treatment may prolong the survival time.