Long-term outcome of XXXX .

@entity5 @entity1 @entity4261 @entity1490
PURPOSE: Extramammary @entity4261 of the perianal region is a rare finding that often results in delayed diagnosis and treatment. Although the natural history of the disease is not well characterized, it historically has been associated with other @entity5 . This study summarizes the history and treatment of all @entity1 diagnosed with @entity4261 at a single institution. METHODS: Charts of all @entity1 with a diagnosis of extramammary @entity4261 of the perianal region confirmed or treated at Memorial Sloan-Kettering Cancer Center between 1950 and 2000 were reviewed. @entity1 with @entity4261 or @entity1490 were excluded except when @entity4261 of the perianal region was diagnosed first. Whenever possible, follow-up information was updated. Estimates of overall and disease-free survival were made by the method of Kaplan and Meier. RESULTS: Twenty-seven @entity1 with a median age of 63 years were diagnosed with @entity4261 . Most @entity1 (74 percent) were treated with wide excision. Local recurrence occurred in 37 percent of all @entity1 treated and in 30 percent of @entity1 (6/20) undergoing a wide excision as part of their treatment. An invasive component was identified in 44 percent of @entity1 (12/27) with @entity4261 . Six @entity1 (22 percent) required a colostomy as part of the treatment for their disease. Adjuvant chemoradiotherapy was used in 22 percent of @entity1 (6/27) who had more aggressive disease. At a median follow-up of 67 months, 56 percent (15/27) had no evidence of disease, and two @entity1 had died of metastatic disease. The overall and disease-free survival at five years was 59 and 64 percent, respectively, which decreased to 33 and 39 percent, respectively, by ten years. CONCLUSIONS: @entity4261 is a rare finding even at a large referral center. The disease process is generally a prolonged one marked by frequent recurrences, and the treatment of first choice is wide excision. @entity1 with @entity5 require more extensive surgery. The role of chemoradiotherapy remains undefined in this disease.
