目的:探讨肛管直肠恶性黑色素瘤流行病学、临床及病理学特征、诊断、鉴别诊断、最新治疗和预后.方法:结合我科收治的1例原发性肛管直肠恶性黑色素瘤伴全身多处转移临床资料,查阅并分析国内外相关文献.结果:ARMM分为无色素性和有色素性肛管直肠恶性黑色素瘤,大多数发生于50岁以上女性,主要临床症状为肛门部肿块或疼痛、便血等,肿瘤距肛缘几乎均<7cm,易误诊为痔疮、直肠癌等常见疾病.免疫组化HMB45、Vimentin、S-100具有临床诊断意义,早期易发生血源性转移或淋巴道转移,肿瘤大小、浸润深度和分期、有无远处转移、手术或治疗方式等因素影响患者的生存率,预后极差.结论:肛管直肠恶性黑色素瘤是一种罕见的皮肤恶性肿瘤,恶性程度高,病因尚不明,误诊率可达80﹪以上.目前首选治疗为手术治疗,生物治疗和免疫治疗为术后首选辅助治疗,早期发现早期诊断早期治疗可以有效降低死亡率.“,”Objective:to investigate the epidemiology、clinical and pathology features、diagnosis、differential diagnosis、the latest treatment method and prognosis of anorectal malignant melanoma.Methods: one case of anorectal malignant melanoma and systemic multiple metastasis was treated in our surgery ward of hospital,reading and analyzing associated literature of ARMM .Results:ARMM consists of Amelanotic and melanotic melanoma,majority of cases occur in women over the age of 50.The common clinical manifestation including sweling or pain of anus、hematochezia and so on.The distance of anal sweling from the anal margin almost <7cm,and easily misdiagnosed as common diseases such as hemorrhoids, rectal cancer.Immunohistochemistry of HMB45, Vimentin, S-100 have clinical significance for the diagnosis of ARMM.Hematogenous and lymphatic metastasis occur at the very earlier stage easily.The diameter of tumor、The depth of invasion and pathology staging, whether distant metastasis or not, operation or treatment means can affect the survival rate.Conclusion:anorectal malignant melanoma is rare skin malignant cancer,at a very high-grade malignant.It is unclear for the pathogeny so far and the misdiagnosis rate more than 80﹪.Surgery is the primary choice for the ARMM currently and biological modality is the preference for adjuvant treatment postoperatively.Yervoy immunotherapy prolong the survival period for unresectable or metastatic ARMM.The overal prognosis is badly poor.Early detection、early diagnosis、early proper treatment is crucial for reducing mortality effectively.