Childhood multifocal osteosarcoma. Clinicopathologic and radiologic correlates.

Abstract

The authors reviewed the clinical charts and pathologic and radiographic materials of nine pediatric patients (mean age, 11.0 years) presenting with multiple skeletal foci of osteosarcoma. Four patients presented with pulmonary metastases, and five had osseous disease only. All had densely sclerotic, predominantly osteoblastic tumors, usually in metaphyseal locations. Evidence of aggressive tumor cell behavior--such as vascular, articular, epiphyseal, or soft-tissue invasion--was usually present in surgical specimens. Despite intensive chemotherapy, all patients died from 6 to 37 months after diagnosis (median survival, 12 months). Comparison of clinical and pathologic features for patients presenting with or without pulmonary metastases failed to disclose any notable differences between the two groups, indicating a common pathogenesis and evolution for their multifocal disease. The authors conclude that this variant of osteosarcoma is a highly aggressive form of the disease that may be accompanied by pulmonary metastases.