Rhabomyosarcoma

Alternative names:  sarcoma botryoides; embryonal cell sarcoma 

Definition:  A highly malignant (cancerous), soft tissue tumor in children. A rhabdomyosarcoma tends to affect 4 major anatomic sites: the head and neck, the genito-urinary tract, the extremities, and the
trunk. 

Causes, incidence, and risk factors:  The cause of rhabdomyosarcoma is unknown. Increased incidence is seen in families with a history of brain tumors and early breast cancer. In young children, the tumor affects all of the 4 major anatomic areas, while in the adolescent it is most common in the genitourinary system. The tumor may appear as a swelling or lump in the soft tissue. There may be no pain. The botryoides type, which is more common in the vagina, generally has the appearance of a grape like cluster and may protrude from the vagina. Diagnosis of rhabdomyosarcoma is often delayed because of lack of symptoms and its appearance may coincide with a recent injury. Early diagnosis is important because rhabdomyosarcoma is an aggressive tumor that metastasizes (spreads) early. 

Symptoms:  Symptoms may vary depending on location of the tumor. Tumors in the vagina may be visible protruding from the opening of the vagina. Bladder and vaginal tumors may cause urinary or bowel obstruction. Tumors in the nose or throat may cause bleeding, congestion, swallowing problems or neurological problems if they extend into the brain. 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. 

Signs and tests: 

  • biopsy, tumor (demonstrates rhabdomyosarcoma)
  • CT scan, head (demonstrates tumor and possible invasion of central nervous system)
  • ultrasound (demonstrates tumor of nasopharynx, throat)
  • cystourethrogram (demonstrates bladder tumors)
  • pelvic examination (direct visualization of the tumor)
  • X-ray chest (may demonstrate metastasis)
  • bone marrow (may demonstrate metastasis)

Treatment: Often rhabdomyosarcoma cannot be removed completely or has already spread when it is discovered. Surgery is the primary form of treatment. Chemotherapy and radiation therapy are used in conjunction with surgery. 

Expectations (prognosis):  Long-term survival does occur. Pulmonary metastasis and widespread diseases are unfavorable prognostic signs. 

Complications: 

  • metastasis 
  • unresectable location 
  • complications from chemotherapy 

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