肛管直肠恶性黑色素瘤(anorectal malignant melanoma，ARMM)是一种非常罕见且预后极差的恶性肿瘤，其发病率低，缺乏特异性临床表现，而且常规病理学检查不易诊断，容易误诊，ARMM的治疗采用外科手术、放疗、化疗及靶向治疗等综合治疗方式，因其对放化疗敏感性较差，因此外科手术切除仍为本病的主要治疗手段。作者通过解读近年来有关ARMM的治疗指南，对该病的生物学特性、诊断和治疗进展进行分析，以期对临床诊治有所裨益。

Anorectal malignant melanoma(ARMM)is a malignant tumor with low incidence and poor prognosis.ARMM lacks specific clinical manifestations, and routine pathological examination is hard to diagnose.The treatment of ARMM includes surgical operation, radiotherapy, chemotherapy and targeted therapy.Surgical operation excision remains the main method to cure the disease because of its poor sensitivity to chemotherapy and radiotherapy.The author analyzed the treatment guidelines in recent years on ARMM, discussed the progress of the diagnosis and biological characteristics, which is helpful for clinical diagnosis and treatment.