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Definition:

Rhabdomyosarcoma is a malignant (cancerous) soft tissue tumor found most often in children. The most common sites are the structures of the head and neck, the urogenital tract, and the arms or legs.



Alternative Names:

Soft tissue sarcoma; Alveolar rhabdomyosarcoma; Embryonal rhabdomyosarcoma; Sarcoma botryoides



Causes, incidence, and risk factors:

The cause of rhabdomyosarcoma is unknown. It is a rare tumor with only several hundred new cases per year throughout the United States. Some children with certain birth defects are at increased risk, and some families have a gene mutation that elevates risk. However, the vast majority of children with rhabdomyosarcoma do not have any known risk factors.



Symptoms:

Symptoms vary depending on location of the tumor. Tumors in the nose or throat may cause bleeding, congestion, swallowing problems, or neurological problems if they extend into the brain.

Tumors in the vagina may be visibly protruding from the opening of the vagina. Bladder and vaginal tumors may cause urinary or bowel obstruction . Tumors in the muscles may appear as a painful lump and are often thought to be an injury. However, they do not go away, but continue to grow, even though they may not continue to be painful.

Diagnosis of rhabdomyosarcoma is often delayed because of lack of symptoms, and because the tumor may appear at the same time as a recent injury. Early diagnosis is important because rhabdomyosarcoma is an aggressive tumor that spreads quickly.



Signs and tests:
  • Biopsy of the tumor
  • CT scan of the chest to look for spread of the tumor
  • CT scan of the tumor site
  • Bone marrow biopsy (may show the cancer has spread)
  • Bone scan to look for spread of the tumor
  • MRI scan of the tumor site
  • Possible spinal tap, to check for spread into the brain, depending on the site of the tumor
  • Thorough physical exam


Treatment:

The precise treatment depends on the site and type of rhabdomyosarcoma. This tumor is best treated at a center with experience treating rhabdomyosarcoma.

In general, surgery and radiation therapy are used to treat the primary site of the tumor, while chemotherapy is used to treat disease at all sites in the body.

Chemotherapy is an essential part of treatment to prevent further spread of the cancer. Many different chemotherapy drugs are active against rhabdomyosarcoma. Some of these drugs include:

  • Vincristine
  • Actinomycin-D
  • Cyclophosphamide (Cytoxan)
  • Doxorubicin (Adriamycin)
  • Melphalan
  • Ifosfamide
  • Etoposide
  • Topotecan


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):

While aggressive treatment is usually necessary, most children with rhabdomyosarcoma will achieve long-term survival. Cure depends on the specific type of tumor, its location, and the amount that has spread.



Complications:
  • Complications from chemotherapy
  • Location in which surgery is not possible
  • Metastasis


Calling your health care provider:

Call your health care provider if your child has symptoms of rhabdomyosarcoma.



Prevention:



References:

Skubitz KM, D'Adama DR. Sarcoma. Mayo Clin Proc. 2007:82(11):1409-1432.

Maki RG. Pediatric sarcomas occurring in adults. J Surg Oncol. 2008:97(4):360-368.




Review Date: 3/21/2008
Reviewed By: Stephen Grund, MD, PhD, Chief of Hematology/Oncology and Director of the George Bray Cancer Center at New Britain General Hospital, New Britain, CT. Review provided byVeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
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