Paget's sarcoma: limb salvage by custom mega prosthesis: four case reports.

Abstract

Of 4 Paget's sarcoma patients (age range, 55-68 years) underwent limb salvage surgery by custom mega prosthesis, 3 had lesions in the upper extremity and one in the proximal femur. Three of the patients were at stage IIB of the disease, according to Enneking's system of staging musculoskeletal tumours. All 4 patients underwent wide resection with a mean length of 152.5 mm. The defects were reconstructed with custom-made prostheses: proximal humeral prostheses in 2 of the patients, total elbow prosthesis in one, and total hip prosthesis in one. During a mean postoperative follow-up period of 40 months, one died of disseminated disease 14 months after surgery; one remained disease-free; 2 had local recurrence and required amputation, of whom one died of disseminated disease one year after amputation, the other had no further evidence of the disease. We report the functional outcomes of the 2 patients who were alive at the latest follow-up. The 2-year patient survival rate was 50%.