Leiomyosarcoma of the rectum is an uncommon malignancy; it accounts for less than 0.1% of all malignancies of the colon and rectum and only one case has been reported long-term following pelvic radiotherapy. We report a second case of leiomyosarcoma of the rectum after radiotherapy in an elderly women who received external radiation therapy eighteen years before, for endometrial carcinoma. We confirm that leiomyosarcoma may be a late uncommon effect of pelvic irradiation and suggest that close and long-term surveillance of irradiated patients is highly recommended.

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