Abstract
Introduction: Types of cancer occurring in children are very different from those occurring in adults. Reliable data on incidence and mortality of childhood cancers is sparse.
Methods: A review of all primary malignant bone tumors in children (0–14 Yrs) from the Scottish National Bone Tumor Registry (1940–2000) is presented. Epidemiology, clinical presentation, pathology, radiological characteristics, treatment options, recurrence rates, geographic distribution and incidence are discussed.
Results: Excluding myelomas and lymphomas, 154 patients were identified. 122 (80.2%) lesions were benign, and 30 (19.7%) malignant. There were 20 osteosarcomas (66%), 8 Ewing’s sarcomas (26%), 1 chondrosarcoma and 1 fibrosarcoma.
Osteosarcoma – Age at presentation ranged from 4–14 Yrs (mean 10.3Yrs). 70 % involved 10–14 Yrs. Male: Female incidence was 1.5:1. 75% of lesions involved the proximal humerus.15 % presented with pathological fractures. The mean cumulative 5 year survival was 20%. Death was usually due to pulmonary metastasis.65% had pulmonary metastasis at a mean 6.3 mts after diagnosis. Mean survival in these patients was 14mts. Survival was superior with adjuvant chemotherapy and wide excision.
Ewing’s Sarcomas- Age at presentation ranged from 7–14 (mean 11.2Yrs).71.4% involved 10–14Yrs. Male: Female was 1.6:1. 62.5% lesions involved the humerus and 25 % the radius and 12.5% the scapula. The mean cumulative 5 year survival was 37.5%.Death was due to pulmonary or skeletal metastasis (mean 21.5mts). All patients had radiotherapy and chemotherapy.
Chondrosarcoma- A rare low-grade chondrosarcoma of the proximal humerus was encountered. Excision and grafting yielded good results.
Discussion: Majority of bone lesions in this age group are benign. Osteosarcomas and Ewing’s sarcomas predominate among the malignant (93%).The peak incidence occurs with adolescent growth spurt. Mean age is lower for osteosarcomas, and the sex incidence for both show male preponderance. Survival rates for Ewing’s was higher than for osteosarcomas. Pulmonary involvement at presentation had worst prognosis.
Correspondence should be addressed to BOOS at the Royal College of Surgeons, 35 - 43 Lincoln’s Inn Fields, London WC2A 3PN
