A neurogenic sarcoma without NF-1 was discovered in a 73-year-old woman in the anorectal region, an unusual site for these tumors. The tumor was of high-grade malignancy and deeply located with mesorectal infiltration; it did not originate from a major nerve. We presume an origin from less differentiated neural crest cells and present a review of the literature on the best treatment for these neoplasms.
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