Unit of Dermatology, IRCCS Ospedale San Raffaele.
Università Vita-Salute, San Raffaele, Milan.
Melanoma Res. 2024 Dec 1;34(6):487-496. doi: 10.1097/CMR.0000000000001003. Epub 2024 Oct 4.
Anorectal melanoma (ARM) is a rare malignancy often associated with a poor prognosis due to its late diagnosis and aggressive biological behavior. This review aims to comprehensively investigate ARM's diagnosis, management, and treatment, emphasizing its clinical characteristics, laboratory findings, and implications for patient prognosis. A systematic literature search was conducted in PubMed, Embase, and Cochrane CENTRAL databases from inception to 1 July 2024. This review synthesizes existing literature to provide a comprehensive understanding of this rare primary malignancy. A total of 110 articles reporting on 166 patients were included. Gender data were available for 131 cases, comprising 67 females (51.1%) and 64 males (48.9%). The median age was 66 years. The overall median time to diagnosis was 4 months for anal melanoma, 3 months for rectal melanoma, and 4 months for anorectal junction melanoma. The clinical presentation was nodular in 98.2% of cases. Pre-diagnosis symptoms included bleeding in 84.9% of cases, mucous elimination (6%), pain (68.7%), tenesmus (16.9%), and changes in bowel movements (28.5%). Overall survival (OS) was reported in 82 cases, with a median OS of 11 months: 11 months for anal melanoma, 7 months for rectal melanoma, and 12 months for anorectal junction melanoma. ARM is a rare and aggressive melanoma subtype often diagnosed at an advanced stage, leading to a poor prognosis. A female predominance was observed, consistent with other mucosal melanomas. Anal melanoma exhibited better progression-free survival, and OS compared to rectal and anorectal junction melanoma.
肛门直肠黑色素瘤(ARM)是一种罕见的恶性肿瘤,由于其诊断较晚且具有侵袭性的生物学行为,预后往往较差。本综述旨在全面探讨 ARM 的诊断、管理和治疗,强调其临床特征、实验室发现及其对患者预后的影响。我们在 PubMed、Embase 和 Cochrane CENTRAL 数据库中进行了系统的文献检索,检索时间截至 2024 年 7 月 1 日。本综述综合了现有文献,旨在全面了解这种罕见的原发性恶性肿瘤。共纳入了 110 篇报道 166 例患者的文章。有 131 例患者的性别数据可用,其中女性 67 例(51.1%),男性 64 例(48.9%)。中位年龄为 66 岁。总体而言,肛门黑色素瘤的中位诊断时间为 4 个月,直肠黑色素瘤为 3 个月,肛门直肠交界处黑色素瘤为 4 个月。98.2%的病例表现为结节状。诊断前的症状包括出血(84.9%)、黏液排出(6%)、疼痛(68.7%)、里急后重(16.9%)和排便习惯改变(28.5%)。82 例患者报告了总生存(OS)情况,中位 OS 为 11 个月:肛门黑色素瘤为 11 个月,直肠黑色素瘤为 7 个月,肛门直肠交界处黑色素瘤为 12 个月。ARM 是一种罕见且侵袭性的黑色素瘤亚型,通常在晚期诊断,导致预后较差。观察到女性患病率较高,与其他黏膜黑色素瘤一致。肛门黑色素瘤的无进展生存期和总生存期均优于直肠和肛门直肠交界处黑色素瘤。