Clinical and radiologic analysis of 13 cases of primary neuroectodermal tumors of bone

Radiographs and clinical charts were reviewed in a series of 13 cases of primary neuroectodermal bone tumors, involving 9 males and 4 females, aged from 3 to 32 years (average: 15 years). The average delay between the onset and diagnosis was 5 months. Fever and other systemic symptoms were present in 6 cases; in 4 cases a fracture was the mode of presentation. Seven patients had metastases (4 involving bone) at the time of presentation. Only one patient is still alive after 5 years. The radiologic appearance is that of an aggressive, poorly demarcated tumor, with cortical destruction, periosteal reaction and soft tissue invasion. Comparison with Ewing sarcoma shows little radiologic or clinical difference, except for a poorer prognosis in neuroectodermal bone tumors. (orig./GDG)