Department of Surgery, Huntsman Cancer Institute at the University of Utah, Salt Lake City, UT 84114, United States.
World J Gastroenterol. 2021 Jan 21;27(3):267-280. doi: 10.3748/wjg.v27.i3.267.
Anorectal melanoma (ARM) is a rare disease with a poor prognosis. Evidence on optimal treatment is limited and surgical management varies widely. We hypothesized that the frequency of abdominoperineal resection used as primary treatment of ARM has decreased over the past several decades.
To update our understanding of outcomes for patients with ARM and analyze management trends around the world.
This is a multi-institutional, retrospective study of patients treated for ARM at 7 hospitals. Hospitals included both large, academic, tertiary care centers and smaller, general community hospitals. Using prospectively maintained institutional tumor registries, we identified 24 patients diagnosed with ARM between January 2000 and May 2019. We analyzed factors prognostic for recurrence and survival. We then used Cox regression to measure overall survival (OS) and melanoma-specific survival. We also performed a literature review to assess trends in surgical management and outcomes.
Of the 24 patients diagnosed with ARM, 12 (50.0%) had local, 8 (33.3%) regional, and 4 (16.7%) distant disease at diagnosis. Median time to recurrence was 10.4 mo [interquartile range (IQR) 7.5-17.2] with only 2 patients (9.3%) not developing recurrence following surgical resection. Median OS was 18.8 mo (IQR 13.5-33.9). One patient is still alive without recurrence at 21.4 mo from diagnosis; no other patient survived 5 years. Primary surgical management with abdominoperineal resection (APR) wide excision (WE) did not lead to differences in OS [hazard ratio = 1.4 (95%CI: 0.3-6.8)]. Review of the literature revealed geographic differences in surgical management of ARM, with increased use of WE in the United States and Europe over time and more frequent use of APR in Asia and India. There was no significant improvement in survival over time.
There is wide variation in the management of ARM and survival outcomes remain poor regardless of approach. Surgical management should aim to minimize morbidity.
肛门直肠黑色素瘤(ARM)是一种预后不良的罕见疾病。目前关于最佳治疗方法的证据有限,手术治疗方法差异很大。我们假设过去几十年中,作为 ARM 主要治疗方法的腹会阴切除术的使用频率有所下降。
更新我们对 ARM 患者的治疗结果的理解,并分析全球的治疗管理趋势。
这是一项在 7 家医院进行的多机构回顾性研究,纳入了接受 ARM 治疗的患者。这些医院包括大型学术性三级护理中心和较小的普通社区医院。通过前瞻性维护的机构肿瘤登记处,我们确定了 2000 年 1 月至 2019 年 5 月期间诊断为 ARM 的 24 名患者。我们分析了与复发和生存相关的预后因素。然后我们使用 Cox 回归测量总生存率(OS)和黑色素瘤特异性生存率。我们还进行了文献复习,以评估手术管理和结果的趋势。
在诊断为 ARM 的 24 名患者中,12 名(50.0%)患者在诊断时患有局部疾病,8 名(33.3%)患者患有区域疾病,4 名(16.7%)患者患有远处疾病。复发的中位时间为 10.4 个月(IQR 7.5-17.2),仅 2 名患者(9.3%)在接受手术切除后未复发。中位 OS 为 18.8 个月(IQR 13.5-33.9)。1 名患者在诊断后 21.4 个月仍无复发且存活;没有其他患者存活 5 年。以腹会阴切除术(APR)广泛切除术(WE)为主的初次手术治疗并没有导致 OS 上的差异[风险比=1.4(95%CI:0.3-6.8)]。文献复习显示,ARM 的手术治疗方法在地理上存在差异,随着时间的推移,美国和欧洲 WE 的使用有所增加,而亚洲和印度 APR 的使用更为频繁。随着时间的推移,生存并未显著改善。
ARM 的治疗管理方法差异很大,无论采用何种方法,生存结果仍然很差。手术管理应旨在最大程度地降低发病率。