Keloids are considered as benign fibroproliferative skin tumors, and rare cases of malignancies have been reported. We present a case of low-grade myofibroblastic sarcoma arising from a recurrent painful keloid scar on the right chest wall after video-assisted thoracic surgery for pneumothorax in a 77-year-old man. Wide composite excision of the keloid, surrounding ribs, and partial diaphragm were performed. The chest wall pleural defect was reconstructed with Teflon (Chemours, Wilmington, DE), and soft tissue was reconstructed with a transverse rectus abdominis myocutaneous flap. This case highlights that refractory keloids may be considered a harbinger of malignancy.
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