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Sarcomatous change in the Pagetoid tibiae
Himanshu Sharma1, E MacDuff, M J Jane
1University Department of Orthopaedic Surgery/Scottish Bone Tumour Registry, Western Infirmary, Glasgow, G11 6NT, UK. hksharma1@aol.com
International Orthopaedics
|August 12, 2005
Summary
Paget's sarcoma of the tibia is rare, often presenting as worsening pain and diagnosed via lytic lesions. Treatment involves limb ablation, with a median survival of 17 months.
Area of Science:
- Orthopedic Oncology
- Skeletal Pathology
Background:
- Paget's sarcoma is a rare malignant bone tumor.
- Tibial involvement is less common but significant.
Purpose of the Study:
- To analyze the characteristics and outcomes of tibial Paget's sarcoma.
- To review cases from the Scottish Bone Tumour Registry.
Main Methods:
- Retrospective review of 13 tibial Paget's sarcoma cases (1947-2004).
- Analysis of presenting features, radiological findings, histology, treatment, and survival.
Main Results:
- 14% of all Paget's sarcoma cases; predominantly affected males.
- Common symptoms: pain, mass, pathological fracture, tibial bowing.
- Radiologically: lytic lesions were prevalent.
- Histology: Osteosarcoma and malignant fibrous histiocytoma were common.
- Treatment: Limb ablation (9 trans-femoral amputations) and adjuvant therapies.
- Median survival: 17 months.
Conclusions:
- Tibial Paget's sarcoma presents with distinct clinical and radiological features.
- Limb ablation and adjuvant therapies are standard treatments.
- Outcomes remain challenging, with limited median survival.