Ewing sarcoma

Ewing sarcoma is a malignant small round blue cell tumor of bone, first described by James Ewing as an endothelioma of bone in 1921. It is the third commonest primary malignant bone tumor in all age-groups following osteosarcoma and multiple myeloma. Among children and young adults, it is the second frequently occurring bone malignancy. Studies have shown lower incidence of this tumour among the African and East Asian population as compared to the Whites with a male preponderance. Most studies in the Asian population are hospital or laboratory based and focussed on treatment outcomes with no recent population based evidence of bone sarcoma incidence from India. The Bombay Cancer Registry, the oldest population based registry in India, had reported Ewing sarcoma as the commonest bone malignancy in the late part of the 90s. The Population based Cancer registry reports of the National Cancer Registry Programme, India provided incidence of all bone malignancies but description by specific histologic types was not presented. This paper provides a detailed analysis of the magnitude of incidence and burden of Ewing sarcoma of bone in the Indian population based on thirty years’ data from the Population based cancer registries (PBCR) of the National Cancer Registry Programme (NCRP), India.

The Ewing family of tumors (Ewing's sarcoma and primitive neuroectodermal tumor [PNET]) is the second most common malignant bone tumor (after osteogenic sarcoma) in children and adolescents. These tumors can also occur in soft tissue, presenting in a manner similar to rhabdomyosarcoma. Nearly half of all patients with Ewing's family tumors are between 10 and 20 years of age, and 70% are under the age of 20, with a slight male predominance. The annual incidence in the United States is 2.1 cases per million children.81 A similar incidence of these tumors occurs throughout the world in countries composed mostly of whites or persons of Hispanic origin; however, these tumors are extremely rare in black children and children of Asian origin.15, 36 The reason for this striking difference in incidence according to racial background is unknown. Ewing's tumor was originally described in 1921 by James Ewing as an endothelioma.14 Over the past decade it has become clear that Ewing's sarcoma, in fact, derives from a primitive neuroectodermal cell with variable differentiation. Classical Ewing's sarcoma is a poorly differentiated small round blue cell tumor, whereas, on the other end of the scale, PNET shows quite discernible differentiation. Both share the same histochemical staining profile and a unique characteristic translocation or a variation of the same within the tumor cell. Virtually all Ewing's tumors contain the chromosomal marker.

Ewing sarcoma is more common in males (1.6 male:1 female) and usually presents in childhood or early adulthood, with a peak between 10 and 20 years of age. It can occur anywhere in the body but most commonly in the pelvis and proximal long tubular bones, especially around the growth plates. The diaphyses of the femur are the most common sites, followed by the tibia and the humerus. Thirty percent are overtly metastatic at presentation. 10-15% of people present with a pathologic fracture at the time of diagnosis. People usually experience extreme bone pain. Rarely, it can develop in the vagina.

Signs and symptoms include intermittent fevers, anemia, leukocytosis, increased sedimentation rate, and other symptoms of inflammatory systemic illness.

According to the Bone Cancer Research Trust (BCRT), the most common symptoms are localized pain, swelling, and sporadic bone pain with variable intensity. The swelling is most likely to be visible if the sarcoma is located on a bone near the surface of the body, but when it occurs in other places deeper in the body, like on the pelvis, it may not be visible.

Feel free to share your research work on related topics in Journal of Orthopedic Oncology which offers information in all aspects of primary, malignant tumors. Osteosarcoma, Ewing's sarcoma, chondrosarcoma, chordoma, and soft tissue sarcomas etc., it also deals with diagnostic methods, therapeutic approaches, clinical, laboratory research and reconstructive techniques.

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Journal Considers Research article, Review articles, editorial, letter, case reports, short communications, original articles from leading scientists and scholars around the world in all areas of related to Malignantosteoid, Multilobular tumour of bone, Chondrosarcoma, Chordoma, Osteosarcoma, Ewing's sarcomaetc., which come under the scope of the journal.

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Regards,

Stella

Editorial Team

Journal of Orthopedic Oncology