Small cell osteosarcoma of bone. Review of 72 cases.

Abstract

Small cell osteosarcoma of bone is a rare form of osteosarcoma, with an incidence rate of 1.3%. This tumor must be differentiated from other small cell malignancies because of treatment considerations, particularly patient response to chemotherapy. Clinicopathologic findings in 72 cases (22 from Mayo Clinic files and 50 from consultation files) of small cell osteosarcoma of bone were studied. The femur was the most common bone involved, although the tumor was found in all portions of the skeleton. Radiographic features (available in 35 cases) suggested a diagnosis of osteosarcoma in 20 cases, Ewing's sarcoma or lymphoma in 14 cases, and giant cell tumor in 1 case. Histologically, there were four types according to the predominant cell size and cytologic features. Osteoid production was identified in all tumors. Complete treatment and follow-up data were available for 45 cases. Generally, in those cases without surgical treatment, greater than 60% of patients died of disease within 2 years. If the surgical procedure was associated with a marginal tumor margin, the prognosis was poor. In the 30 patients with wide or radical surgical margins, at last follow-up 13 were alive with no evidence of disease, 2 were alive with disease, and 15 died of disease at 5 months to 13.1 years after diagnosis. In 16 of 22 Mayo Clinic patients, excluding those who presented with metastasis, the cumulative 5-year survival rate was 28.9%. Median survival time in patients who had surgery with additional chemotherapy was 13.4 years, compared with 1.4 years in patients who underwent surgery alone (P = 0.17). Small cell osteosarcoma is a definite reproducible histologic entity. Treatment should be based on a protocol for osteosarcoma.