Abstract
Malignant primary rectal melanomas (PRM) are rare tumors. Their diagnosis is frequently delayed as these lesions are often mistaken for benign diseases, resulting in extremely poor overall survival. Histological evaluation with special immunohistochemical (IHC) stains is often indispensable for a definitive diagnosis. The main treatment for this condition involves surgical resection. Adjuvant therapy has also been long recommended. We discuss the case of a 60-year-old woman who presented with changes in bowel habits, anal pain, and perineal burning with no bleeding. A digital rectal examination revealed a nodular mass extending 5 cm from the anal verge. Rectosigmoidoscopy demonstrated an ulcerated polypoid tumor extending 4 cm from the anal verge and over 5 cm into the lower rectum. Biopsy and IHC tests confirmed the diagnosis of rectal melanoma. The patient was successfully managed with surgery followed by external beam radiotherapy and a complete response was achieved after 10 months of follow-up.