Ewing's sarcoma
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| Ewing's sarcoma Classification and external resources | |
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| X-Ray of a child with Ewing's sarcoma of the tibia | |
| ICD-9 | 170.9 |
| ICD-O: | 9260/3 |
| DiseasesDB | 4604 |
| MedlinePlus | 001302 |
| eMedicine | ped/2589 |
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US National Guidelines Clearinghouse on Ewing's sarcoma NICE Guidance on Ewing's sarcoma
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Overview
Ewing's sarcoma is the common name for primitive neuroectodermal tumor. It is a rare disease in which cancer cells are found in the bone or in soft tissue. The most common areas in which it occurs are the pelvis, the femur, the humerus, and the ribs. James Ewing (1866-1943) first described the tumor, establishing that the disease was separate from lymphoma and other types of cancer known at that time. Ewing's sarcoma occurs most frequently in male teenagers.
Ewing's sarcoma is the result of a translocation between chromosomes 11 and 22, which fuses the EWS gene of chromosome 22 to the FLI1 gene of chromosome 11.
Epidemiology
The frequency in the United States depends on the patient's age, with a rate of 0.3 case per 1,000,000 children in those younger than 3 years of age to as high as 4.6 cases per 1,000,000 in adolescents aged 15-19 years. Internationally the annual incidence rate averages less than 2 cases per 1,000,000 children.[1] In the United Kingdom an average of six children per year are diagnosed, mainly males in early stages of puberty.
Due to the prevalence of diagnosis during teenage years, there may possibly be a link between the onset of puberty and the early stages of this disease, although no research is currently being conducted to confirm this theory.
Clinical findings
Ewing's sarcoma is more common in males 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. The diaphyses of the femur are the most common sites, followed by the tibia and the humerus. Thirty percent are overtly metastatic at presentation.
The most common clinical findings are pain and swelling diarrhea.
Imaging findings
On conventional radiographs, the most common osseous presentation is a permeative lytic lesion with periosteal reaction. 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.g. permeative) is the most useful plain film characteristic in differention of benign versus aggressive or malignant lytic lesions.
MRI should be routinely used in the work-up of malignant tumors. MRI will show the full bony and soft tissue extent and relate the tumor to other nearby anatomic structures (e.g. vessels). Gadolinium contrast is not necessary as it does not give additional information over non contrast 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.
CT can also be used to define the extraosseous extent of the tumor, especially in the skull, spine, ribs and pelvis. Both CT and MRI can be used to follow response to radiation and/or chemotherapy.
Bone scintigraphy can also be used to follow tumor response to therapy.
Differential diagnosis
Other entities that may have a similar radiologic presentation include osteomyelitis, osteosarcoma (especially telangiectatic osteosarcoma) and eosinophilic granuloma. Soft tissue neoplasms such as malignant fibrous histiocytoma that erode into adjacent bone may also have a similar appearance.
Treatment
Because almost all patients with apparently localized disease at diagnosis have occult metastatic disease, multi drug chemotherapy as well as local disease control with surgery and/or radiation is indicated in the treatment of all patients. Treatment often consists of neo-adjuvant chemotherapy generally followed by wide or radical excision, and may also include radiotherapy. Complete excision at the time of biopsy may be performed if malignancy is confirmed at that time. Treatment lengths vary depending on location and stage of the disease at diagnosis. Radical chemotherapy may be as short as 6 treatments at 3 week cycles, however most patients will undergo chemotherapy for 6-12 months and radiation therapy for 5-8 weeks.
Prognosis
Staging attempts to distinguish patients with localized from those with metastatic disease. Most commonly, metastases occur in the chest, bone and/or bone marrow. Less common sites include the central nervous system and lymph nodes.
Survival for localized disease is 65-70% when treated with chemotherapy. Long term survival for metastatic disease can be less than 10% but some sources state it is 25-30%.
References
- ↑ http://www.emedicine.com/radio/topic275.htm eMedicine article on Ewing's sarcoma accessed April 24, 2006
External links
- National Cancer Institute listing for Ewing's sarcoma
- Ewing's Research Foundation
- Ewings Sarcoma Family of Tumors (ESFT) by R. Lor Randall, MD
- Adult Bone Cancer Survivors - Ewing's sarcoma survivors' stories, resources, and support forum
- Liddy Shriver Sarcoma Initiative
- Natural History of Ewings Sarcoma
- Mark Cross Bone Cancer Fund [3]
Acknowledgement and Attribution Regarding Sources of Content
Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .
