We present a mucosal Schwann cell hamartoma that was observed in a 63-year-old woman with no personal or family history of interest who underwent a screening colonoscopy for early detection of colon cancer. During colonoscopy, a small polyp with a maximum diameter of 0.8 cm was detected at the rectosigmoid level. Histologically, a benign lesion of neuroid aspect was observed, consisting of multiple spindle cell fascicles that was positive for S-100 and negative for enolase, synaptophysin, CD34, CD117, actin, desmin, Dog1, EMA, and neurofilaments. These findings, supported by the family and personal history of the patient, allowed us to make a diagnosis, discarding other entities.
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