Primitive Neuroectodermal Tumors (PNET)

Alternative names
Ewing’s Family of Tumors; Ewing’s sarcoma

Definition
Ewing’s sarcoma is a malignant (cancerous) bone tumor which affects children.

Causes, incidence, and risk factors

Ewing ‘s sarcoma can occur any time during childhood, but usually develops during puberty, when bones are growing rapidly. It is uncommon in African-American, African, and Chinese children.

The tumor may arise anywhere in the body, usually in the long bones of the arms and legs, the pelvis, or the chest. It may also develop in the skull or the flat bones of the trunk.

There are few symptoms. The most common is pain and occasionally swelling at the site of the tumor. Children may also break a bone at the site of the tumor after a seemingly minor trauma (pathologic fracture). Fever may also be present.

The tumor often spreads (metastasis) to the lungs and other bones. Metastasis is present in approximately one-third of children with this condition at the time of diagnosis.

Symptoms

     
  • Pain at the site of the tumor  
  • Swelling at the site of the tumor (occasionally)  
  • Fever (can occur)

Signs and tests
If a tumor is suspected, tests to locate the primary tumor and any spread (metastasis) often include:

     
  • Skeletal X-ray  
  • Chest X-ray  
  • CT scan of the chest  
  • Bone scan  
  • Biopsy of the tumor

Treatment
Treatment should be done by a cancer specialist (oncologist) and often includes a combination of:

     
  • Chemotherapy       o Cyclophosphamide       o Vincristine       o Dactinomycin       o Doxorubicin       o Ifosfamide       o Etoposide  
  • Radiation therapy to the tumor site  
  • Surgical excision (removal) of the primary tumor

Support Groups
The stress of illness can often be helped by joining a support group where members share common experiences and problems. See cancer - support group.

Expectations (prognosis)
The prognosis depends on the location of the tumor, and whether or not the cancer has spread. The best chance for cure is with a combination of treatments that includes chemotherapy plus radiation or surgery provided at an institution that frequently treats this type of cancer.

Complications
The treatments needed to fight this disease have many complications which should be discussed on an individual basis.

Calling your health care provider
Call your health care provider if your child has symptoms suggestive of Ewing ‘s sarcoma. Early diagnosis can increase the possibility of a favorable outcome.

Johns Hopkins patient information

Last revised: December 3, 2012
by Martin A. Harms, M.D.

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