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Paget's sarcoma: limb salvage by custom mega prosthesis: four case reports.
N Mayilvahanan1, J C Bose, M Paraskumar
1Madras Medical College, Government General Hospital, Chennai, India. drmayil@bonetumour.org
Journal of Orthopaedic Surgery (Hong Kong)
|December 29, 2004
Summary
Limb salvage surgery using custom mega prostheses for Paget's sarcoma showed a 50% 2-year survival rate. Functional outcomes were reported for surviving patients, with 2 requiring amputation due to recurrence.
Area of Science:
- Orthopedic Oncology
- Surgical Reconstruction
- Musculoskeletal Tumors
Background:
- Paget's sarcoma is a rare malignant bone tumor.
- Limb salvage surgery aims to preserve function while removing tumors.
- Custom mega prostheses offer solutions for extensive bone defects.
Observation:
- Four patients with Paget's sarcoma (ages 55-68) underwent limb salvage with custom mega prostheses.
- Lesions were in the upper extremity (3) and proximal femur (1), all at Enneking stage IIB.
- Mean resection length was 152.5 mm, with reconstructions using proximal humeral, total elbow, or total hip prostheses.
Findings:
- The 2-year patient survival rate was 50%.
- One patient died of disseminated disease; one remained disease-free.
- Two patients experienced local recurrence requiring amputation; one of these later died of disseminated disease.
Implications:
- Custom mega prostheses can be utilized in limb salvage for Paget's sarcoma.
- Surgical outcomes demonstrate a significant challenge with local recurrence and survival.
- Further research is needed to optimize treatment strategies for this rare condition.