Alveolar rhabdomyosarcoma

Alternative names
Soft tissue sarcoma; Rhabdomyosarcoma; Embryonal rhabdomyosarcoma; Sarcoma botryoides

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

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 its appearance may coincide with a recent injury. Early diagnosis is important because rhabdomyosarcoma is an aggressive tumor that metastasizes (spreads) quickly.

Signs and tests

• Biopsy tumor (demonstrates rhabdomyosarcoma)
  
• CT scan and/or MRI scan of the tumor site
  
• CT scan of the chest to look for metastases (spread of tumor)
  
• Bone scan to look for metastases (spread of tumor)
  
• Bone marrow biopsy (may demonstrate metastasis)
  
• 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 it has spread.

Complications

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

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

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