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Ewing's Sarcoma

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 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. more...

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Clinical Findings

Ewing's sarcoma usually presents in childhood or early adulthood, with a peak between 10 and 20 years of age, although it can occur in younger children and older adults. It can occur anywhere in the body, but most commonly in the pelvis and proximal long tubular bones. The metaphysis and diaphysis 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.

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 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.

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, multidrug chemotherapy as well as local disease control with surgery and/or radiation is indicated in the treatment of all patients (2). Treatment often consists of 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.

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.

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Doxorubicin with or without ifosfamide in treating patients with locally advanced or metastatic soft tissue sarcoma - Clinical Trial Review
From Journal of Drugs in Dermatology, 8/1/03

Sponsored by: EORTC Soft Tissue and Bone Sarcoma Cooperative Group

RATIONALE: Drugs used in chemotherapy such as doxorubicin and ifosfamide use different ways to stop tumor cells from dividing so they stop growing or die. It is not yet known whether doxorubicin is more effective with or without ifosfamide in treating soft tissue sarcoma.

PURPOSE: Randomized phase III trial to compare the effectiveness of doxorubicin with or without ifosfamide in treating patients who have locally advanced or metastatic soft tissue sarcoma.

Study Type: Interventional

Study Design: Treatment

OBJECTIVES:

* Compare the progression-free and overall survival of patients with locally advanced or metastatic soft tissue sarcoma treated with doxorubicin with vs. without ifosfamide as first-line therapy.

* Compare the response in patients treated with these regimens.

* Compare the treatment-related mortality of patients treated with these regimens.

* Compare the toxicity of these regimens in these patients.

OUTLINE: This is a randomized, open-label, multicenter study. Patients are stratified according to WHO performance status (0 vs. 1), age group (less than 50 years of age vs. 50 years of age and over), presence of liver metastases (yes vs. no), histological grade (2 vs. 3), and participating center. Patients are randomized to 1 of 2 treatment arms.

* Arm I: Patients receive doxorubicin IV on day 1.

* Arm II: Patients receive doxorubicin IV on days 1-3 and ifosfamide IV over 4 hours on days 1-4. In both arms, treatment repeats every 3 weeks for up to 6 courses in the absence of disease progression or unacceptable toxicity. * Patients are followed every 8 weeks until disease progression and then every 12 weeks thereafter.

Ages eligible for study: 18 years - 60 years, both genders

DISEASE CHARACTERISTICS:

* Histologically confirmed soft tissue sarcoma

* Locally advanced unresectable * OR metastatic disease

* High-grade (grade 2-3) disease according to the FNLCC grading system

NOTE: * Disease that could prove resectable (including pulmonary metastasectomy) after a response to chemotherapy is allowed

* The following tumor types are eligible:

* Malignant fibrous histiocytoma

* Myxoid and round cell liposarcoma, pleomorphic liposarcoma, or dedifferentiated liposarcoma

* Pleomorphic rhabdomyosarcoma

* Synovial sarcoma

* Myxofibrosarcoma, intermediate and high-grade

* Fibrosarcoma

* Leiomyosarcoma

* Angiosarcoma

* Malignant peripheral nerve sheath tumor

* Epithelioid sarcoma

* The following tumor types are not eligible:

* Gastrointestinal stromal tumor

* Uterine leiomyosarcoma

* Mixed mesodermal tumor

* Chondrosarcoma

* Malignant mesothelioma

* Neuroblastoma

* Osteosarcoma

* Ewing's sarcoma/primitive neuroectodermal tumor

* Desmoplastic small round cell tumor

* Embryonal rhabdomyosarcoma

* Alveolar soft part sarcoma

* Must have a measurable lesion with clinical evidence of progression within the past 6 weeks

* Osseous lesions and pleural effusions are not considered measurable

* No known or symptomatic CNS metastases

Patient Characteristics:

Performance status

* WHO 0-1

Hematopoietic

* Absolute neutrophil count at least 2,000/[mm.sup.3]

* Platelet count at least 100,000/[mm.sup.3]

Hepatic

* Bilirubin no greater than 1.8 mg/dL

* Albumin at least 2.5 g/dL

Renal

* Creatinine no greater than 1.4 mg/dL OR

* Creatinine clearance greater than 65 mL/min

Cardiovascular

* LVEF normal by echocardiography or MUGA

* No history of cardiovascular disease

Other

* Not pregnant

* Negative pregnancy test

* Fertile patients must use effective contraception

* No other severe medical illness

* No psychosis

* No other prior or concurrent malignancy except adequately treated carcinoma in situ of the cervix or basal cell skin cancer

* No psychological, familial, sociological, or geographical condition that would preclude study compliance and follow-up schedule

PRIOR CONCURRENT THERAPY:

Chemotherapy

* No prior chemotherapy for advanced or metastatic disease

* Prior adjuvant chemotherapy allowed provided there was no disease progression within 6 months after completion of treatment

Radiotherapy

* No prior radiotherapy to the sole index lesion

Location and Contact Information:

Institute of Cancer Research--UK, Sutton, England, SM2 5NG, United Kingdom; Ian Robert Judson, MA, MD, FRCP Study Chair, National Cancer Research Institute (NCRI) Tel: 44-208-722-4302

Denmark United Kingdom, England

A total of 450 patients will be accrued for this study within 4 years

Study ID Numbers CDR0000302584; EORTC-62012

NLM Identifier NCT00061984

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