Ewing sarcoma is a type of tumor that forms in bone or soft tissue. Undifferentiated round cell sarcoma may also occur in the bone or soft tissue. Signs and symptoms of Ewing sarcoma include swelling and pain near the tumor. Tests that examine the bone and soft tissue are used to diagnose and stage Ewing sarcoma.
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The classic description of lamellated or "onion-skin" type periosteal reaction is often associated with this lesion. Plain films add valuable information in the initial evaluation or screening. The wide zone of transition e. It will show the full bony and soft tissue extent and relate the tumor to other nearby anatomic structures e.
Gadolinium contrast is not necessary as it does not give additional information over noncontrast studies, though some current researchers argue that dynamic, contrast-enhanced MRI may help determine the amount of necrosis within the tumor, thus help in determining response to treatment prior to surgery. Computed axial tomography CT can also be used to define the extraosseous extent of the tumor, especially in the skull, spine, ribs, and pelvis.
Bone scintigraphy can also be used to follow tumor response to therapy. These tumors may be accompanied by a large soft-tissue mass while almost no bone destruction is visible. The radiographs frequently do not shown any signs of cortical destruction. Differential diagnosis[ edit ] Other entities with similar clinical presentations include osteomyelitis , osteosarcoma especially telangiectatic osteosarcoma , and eosinophilic granuloma.
Soft-tissue neoplasms such as pleomorphic undifferentiated sarcoma malignant fibrous histiocytoma that erode into adjacent bone may also have a similar appearance. Complete excision at the time of biopsy may be performed if malignancy is confirmed at the time it is examined. Radical chemotherapy may be as short as six treatments at 3-week cycles, but most people undergo chemotherapy for 6—12 months and radiation therapy for 5—8 weeks.
The tumor has a unique property of being highly sensitive to radiation, sometimes acknowledged by the phrase "melting like snow", but the main drawback is that it recurs dramatically after some time. Less common sites include the central nervous system and lymph nodes. Internationally, the annual incidence rate averages less than 2 cases per million children.
Due to the prevalence of diagnosis during teenage years, a link may exist between the onset of puberty and the early stages of this disease, although no research confirms this hypothesis. All three children were diagnosed in and all attended the same temporary classroom together while the school underwent renovation. A fourth teenager living nearby was diagnosed in The odds of this grouping are considered significant.
Ewingov sarkóm: príčiny, príznaky, diagnostika a liečba