摘要
BACKGROUND 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.AIM To update our understanding of outcomes for patients with ARM and analyze management trends around the world.METHODS 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.RESULTS 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)vs 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.CONCLUSION There is wide variation in the management of ARM and survival outcomes remain poor regardless of approach.Surgical management should aim to minimize morbidity.
