A primary tumour in the bone that may occur in any bone but is most prevalent in the shafts of the long bones of the lower extremities, never in the growth joints, and never exhibiting osteoblastic properties. Intermittent pain in any bone, most frequently the jaws, shafts of long bone and pelvis, increasing with progress of disease. The skull, clavicle, ribs, and shoulder and pelvic girdles may be involved. Slight fever. The tumour is composed of compact, uniform cells with large, round, or ovoid nuclei containing scattered chromatin. Miotic changes are common; small vascular channels may be present.
The sarcoma is very destructive and soon will spread to other parts of the body. Even with radical treatment the death rate is very high. Most commonly affected are children, adolescents, and young adults. Ewing's sarcoma accounts for 10-15% of all primary malignant bone tumours. Incidence is about 0.6 per million, less common in black and Chinese populations. Te origin of Ewing's Tumour still remains unclear.
Similar tumours had been reported earlier by Georg Albert Lücke (1829-1894) in 1866 and Otto Hildebrand (1858-1927) in 1890, but it was the work of Ewing which established that the disease was separate from lymphoma or neuroblastoma. Ewing's first paper on "his" sarcoma was first presented at a meeting of the New York Pathological Society in 1920.
- J. Ewing:
Diffuse endothelioma of bone.
Proceedings of the New York Pathological Society, 1921, 17-24. Review and classification of bone sarcoma.
Archives of Surgery, Chicago, 1922, 4: 485-533. Further report on endothelial endothelioma of bone.
Proceedings of the New York Pathological Society, 1924, 24: 93-101.