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. In the 10 patients who died as a direct result of the tumor, death occured on average 8 months following diagnosis if metastases were present initially, and 36 months after the diagnosis otherwise. Tumors predominantly involved the leg (7 cases), the pelvis (2 cases), and the humerus (2 cases), the involvement being both diaphyseal and metaphyseal. 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. Both bone tumors may have a similar neuroectodermal origin, with Ewing sarcoma representing the undifferentiated variety.