Rousselin B, Vanel D, Terrier-Lacombe M J, Istria B J, Spielman M, Masselot J
Department of Radiology, Institut Gustave Roussy, Villejuif, France.
Skeletal Radiol. 1989;18(2):115-20. doi: 10.1007/BF00350659.
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.
回顾了13例原发性神经外胚层骨肿瘤患者的X线片和临床病历，其中男性9例，女性4例，年龄3至32岁（平均15岁）。发病至诊断的平均间隔时间为5个月。6例患者出现发热及其他全身症状；4例患者以骨折为首发表现。7例患者就诊时已有转移（4例累及骨骼）。5年后仅1例患者仍存活。在因肿瘤直接死亡的10例患者中，若最初即有转移，平均在诊断后8个月死亡；若无转移，则在诊断后36个月死亡。肿瘤主要累及腿部（7例）、骨盆（2例）和肱骨（2例），累及骨干和干骺端。X线表现为侵袭性、边界不清的肿瘤，伴有皮质破坏、骨膜反应和软组织侵犯。与尤因肉瘤相比，除神经外胚层骨肿瘤预后较差外，在影像学或临床方面差异不大。这两种骨肿瘤可能有相似的神经外胚层起源，尤因肉瘤代表未分化型。
