X-Ray of a child with Ewing's sarcoma of the tibia
Find information on thousands of medical conditions and prescription drugs.

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...

Home
Diseases
A
B
C
D
E
Ebola hemorrhagic fever
Ebstein's anomaly
Eclampsia
Ectodermal Dysplasia
Ectopic pregnancy
Ectrodactyly
Edwards syndrome
Ehlers-Danlos syndrome
Ehrlichiosis
Eisoptrophobia
Elective mutism
Electrophobia
Elephantiasis
Ellis-Van Creveld syndrome
Emetophobia
Emphysema
Encephalitis
Encephalitis lethargica
Encephalocele
Encephalomyelitis
Encephalomyelitis, Myalgic
Endocarditis
Endocarditis, infective
Endometriosis
Endomyocardial fibrosis
Enetophobia
Enterobiasis
Eosinophilia-myalgia...
Eosinophilic fasciitis
Eosophobia
Ependymoma
Epicondylitis
Epidermolysis bullosa
Epidermolytic hyperkeratosis
Epididymitis
Epilepsy
Epiphyseal stippling...
Epistaxiophobia
EPP (erythropoietic...
Epstein barr virus...
Equinophobia
Ergophobia
Erysipelas
Erythema multiforme
Erythermalgia
Erythroblastopenia
Erythromelalgia
Erythroplakia
Erythropoietic...
Esophageal atresia
Esophageal varices
Esotropia
Essential hypertension
Essential thrombocythemia
Essential thrombocytopenia
Essential thrombocytosis
Euphobia
Evan's syndrome
Ewing's Sarcoma
Exencephaly
Exophthalmos
Exostoses
Exploding head syndrome
Hereditary Multiple...
Hereditary Multiple...
Hereditary Multiple...
Hereditary Multiple...
F
G
H
I
J
K
L
M
N
O
P
Q
R
S
T
U
V
W
X
Y
Z
Medicines

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.

Read more at Wikipedia.org


[List your site here Free!]


Ewing's sarcoma tied to other cancers - Brief Article
From Science News, 4/30/94 by Kathy A. Fackelmann

Families with a history of Ewing's sarcoma, a rare type of soft- tissue cancer, develop more than their share skin, brain, and stomach cancers as well, according to a new study.

Cancer researcher Biljana Novakovic and her colleagues at the National Cancer Institute (NCI in Bethesda, Md., studied 256 families with wne member afflicated with EwiNg's sarcoma who had been treated at NCI between 1965 and 1992. The scientists gathered medical information aboDt other members of these families and noted whether any relative had recieved a cancer diagnosis of any type. The NCI researchers compared the observed rate of cancer in those families with rates derived from cancer cases reported to a tumor registry.

They found higher-than-expected rates of melanoma - a dealy skin cancer - brain malignancies, and stomach cancer in the families. The study found no heightened risk of other cancers for relatives of the Ewing's sarcoma patients.

These findings are preliminary , cautions NCI's Margaret A. Tucker. And while the higher-than-average cancer risk seen in families of Ewing's patients may be inherited, a genetic mechanism has yet to be found. However, a single gene probably can't account for the clusters of cancers seen in these families, Novakovic adds.

Although scientists have long believed that Ewing's sarcoma is a cancer of the bone, recent research has shown that it develops in tissue which can be traced back to the so-called neural crest region of the embryo. The brain and skin cancers afflicting these families also arise from tissue derived from the neural crest. Future study may provide researchers with a better understanding of such cancers as well as yield clues about embryonic development, Tucker says.

COPYRIGHT 1994 Science Service, Inc.
COPYRIGHT 2004 Gale Group

Return to Ewing's Sarcoma
Home Contact Resources Exchange Links ebay