原发性肝脏恶性黑色素瘤 (primary liver malignant melanoma, PLMM) 是一种侵袭性强、恶性程度高、预后极差的罕见恶性肿瘤.临床报道显示, 该病以腹胀、腹痛、纳差、恶心、呕吐等临床表现为主, 特异性差, 且目前国内外对该病发病机制、诊断、治疗方案及预后影响因素的研究尚未形成共识.为加深临床医师对PLMM的理解和认识, 更好地解决临床问题, 本文就近年来原发性肝脏恶性黑色素瘤诊疗相关进展做如下综述.“,”Primary liver malignant melanoma (PLMM) is a rare malignant tumor with high aggressive, high degree of malignancy and poor prognosis. Clinical reports showed that the disease was mainly characterized by clinical manifestations such as abdominal distension of abdominal distension and nausea and vomiting. At present, there is no consensus on the pathogenesis, diagnosis, treatment model and prognostic factors. In order to deepen the understanding of PLMM and solve the clinical problems better, this paper reviews the recent advances of the related diagnosis and treatment progress in primary malignant melanoma of the liver.