Ewing's sarcoma
| Ewing's sarcoma | |
| ICD-9 | 170.9 |
|---|---|
| ICD-O: | 9260/3 |
| DiseasesDB | 4604 |
| MedlinePlus | 001302 |
|
Ewing's sarcoma Microchapters |
|
Diagnosis |
|---|
|
Treatment |
|
Case Studies |
|
Ewing's sarcoma On the Web |
|
American Roentgen Ray Society Images of Ewing's sarcoma |
For patient information click here. Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Assistant Editor(s)-In-Chief: Michael Maddaleni, B.S.
Overview
Historical Perspective
Pathophysiology
Epidemiology and Demographics
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%.