OSTEOGENIC SARCOMAS OF THE UPPER LIMB A REVIEW OF THE SCOTTISH BONE TUMOR REGISTRY

Abstract

Background Osteosarcoma is the most common bone sarcoma, and the 3rd most common malignancy in children and adolescents. It accounts for 20% of primary malignant bone tumors.

Methods A retrospective review of osteosarcomas from the Scottish National Bone Tumor Registry (1940–2000) involving the upperlimb bones is presented. Patient demography, type and location of lesions, treatment options, recurrence and survival rates, and metastasis have been analysed.

Results 75 cases were identified from the registry. Sex incidence showed a slight male preponderance with male: female ratio 1.14: 1.Age at presentation ranged from 4–88 Yrs (mean 28.44 Yrs). 46.7% sarcomas occurred in the second decade (11–20 Yrs). The humerus was the bone most frequently involved (78.6% of lesions), and the proximal humerus the commonest site (60%). The scapula was involved in 9.3% and the forearm in 8%.A rare solitary lesion of the clavicle was encountered.17% presented with pathological fractures at diagnosis. Patients typically present with dull aching pain of weeks to months. All patients underwent radiological studies and diagnostic biopsy. Treatment modalities included amputation, limb-sparing surgery, adjuvant/neoadjuvant chemotherapy and radiotherapy. The cumulative 5 year survival for the series was 32%.Death was usually due to pulmonary and skeletal metastasis, and the mean survival in such patients was 21.5 mts. Patients presenting with metastatic pulmonary disease had poor prognosis. Limb-sparing surgery with wide margins does not compromise survival. Results with custom endoprosthesis are encouraging.

Discussion Osteosarcomas require a multidisciplinary approach to diagnosis and treatment to optimise survival. During the first half of the study period amputation was the mainstay of treatment with high incidence of mortality due to metastatic disease. Recent advances in neoadjuvant/adjuvant chemotherapy have improved the ability to perform limb –sparing resections, and disease free and overall survival rates have improved. Regular, long follow-up is indicated in these patients.