INTRODUCTION: @entity5 are increasing in frequency for unknown reasons. Surgery is the principal treatment, and the role of adjuvant therapy has not been defined. We therefore decided to review the experience of the Princess Margaret Hospital in Toronto, a large tertiary care @entity5 hospital, with respect to the surgical management of @entity320 . METHODS: We reviewed the charts of all registered @entity1 with @entity320 ( @entity320 ) treated with surgery or radiotherapy, or both, at the hospital between 1980 and 1999, paying particular attention to survival, and local and distant recurrences. RESULTS: There were 14 @entity1 , all of whom were followed up to the time of @entity204 or for a minimum of 28 months for surviving @entity1 . The mean ages at diagnosis were 56 years for @entity1 and 68 years for @entity1 . Clinical staging was as follows: local, 10 @entity1 ; locoregional, 3 @entity1 and metastatic disease, 1 @entity1 . Local therapy included local resection alone in 7 cases and abdominoperineal resection in 7. Seven @entity1 received pelvic irradiation at some time during their disease, using different doses and fractionation schemes. Three of them had concomitant chemotherapy and radiotherapy with no @entity5 regression. In all 3 @entity1 the lesions was reclassified as @entity320 and the @entity1 underwent surgery. The other 4 @entity1 had a short course of radiotherapy for palliation after the original lesion recurred. The overall median survival was 12 (range from 3-51) months. Two @entity1 remained alive at last follow-up. @entity1 managed by local excision had a median survival of 12 (range from 3-51) months, and those managed by abdominoperineal resection had a median survival of 7 (range 5-51) months. Of the 10 @entity1 treated initially with local excision, 6 required reoperation. Three underwent salvage abdominoperineal resection. Six @entity1 were alive 1 year after treatment (median survival 32.5 mo [range from 21-51 mo]). Eight @entity1 had a rapid evolution of their disease with a median survival of 5.5 (range from 3-12) months. Eleven of the 12 @entity1 who died had metastatic disease. CONCLUSIONS: Systemic dissemination is almost universal in @entity1 with @entity320 . The overall survival was poor regardless of local treatment. There was a 60% failure rate of local excision, which necessitated further surgery. Improving local control is important since some @entity1 will survive up to 3 years.

XXXX : treatment with surgery or radiation therapy, or both.

@entity5 :: ('MESH:D009369', 'Disease') :: ['tumour', 'cancer', 'Anorectal malignant tumours'] @entity1 :: ('9606', 'Species') :: ['women', 'Patients', 'patients', 'patient', 'men'] @entity204 :: ('MESH:D003643', 'Disease') :: ['death'] @entity320 :: ('MESH:D008545', 'Disease') :: ['anorectal malignant melanoma', 'AMM', 'anorectal melanoma']
@entity320 :: (MESH:D008545,Disease) :: ['Anorectal malignant melanoma']
