RHABDOMYOSARCOMA

Alveolar Rhabdomyosarcoma; Embryonal Rhabdomyosarcoma; Sarcoma Botryoides

Rhabdomyosarcoma is a cancerous tumor of unknown cause that occurs mostly in children and teenagers. It grows in the soft tissues of the body, particularly in the muscles that attach to bone and help the body to move. The most common sites for this tumor include the head, neck, bladder, vagina, arms, leg, and trunk. Rhabdomyosarcoma can also be found in places where skeletal muscles are absent or very small, such as in the prostate, middle ear and bile duct system. Symptoms include tumors that may lead to bleeding, congestion, swallowing problems, neurological problems, eye and vision problems, urination or bowel problems, and movement abnormalities.

Determining the severity of the condition is based on clinical groups and stages. There are 4 clinical groups:

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  I,

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  II,

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  III, and

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  IV.

Groups I, II, and III may have evidence of spread to lymph nodes but none to distant sites. Group IV has spread to lymph nodes and distant sites.

There are 4 stages: Stages I, and II, do not have evidence of spread to lymph nodes or distant sites. Stage III has local lymph node involvement and Stage IV has spread to distant lymph nodes or distant sites.

Group IV, Stage IV is the most severe. It has the lowest survival rate. Rhabdomyosarcoma is much more common in children than adults.

Diagnostic testing: Diagnosis is often delayed because of a lack of symptoms or physical findings, and because the tumor may appear at the same time as a recent injury. A complete physical examination should be done.

Tests include:

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  Biopsy of tumor;

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  X-rays;

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  CT scan of tumor site and chest (to look for spread of tumor);

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  PET scan; bone scan;

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  Bone marrow biopsy;

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  MRI of tumor site;

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  Blood and urine tests; and

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  Spinal tap (lumbar puncture) to check the cerebrospinal fluid.

Physical findings: Symptoms of rhabdomyoscarcoma may include:

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  Headache;

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  A tumor or mass that can be seen or felt (may or may not be painful);

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  Bleeding from the nose, ears, vagina, rectum, or throat (may occur if the location of the tumor is in these areas);

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  Tingling, numbness, pain, and movement may be affected if the tumor compresses nerves in the area;

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  Protrusion of the eye or a drooping eyelid (may indicate a tumor behind the eye);

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  Trouble urinating and blood in the urine; and

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  Difficulty with bowel movements.

ICD-9: 171.9

Rhabdomyosarcoma usually occurs in two distinct groups: children age five and under, and adolescents 14-20 years of age. Generally, children with Stage IV rhabdomyosarcoma have a 5-year survival rate of 20 to 25%. Survival rates for Stages I, II, and III are much higher (60 to 90%). While aggressive treatment is usually necessary, most children with rhabdomyosarcoma will achieve long-term survival. Treatment may include surgery, chemotherapy, radiation, and stem cell (bone marrow) transplant.

The specific type of tumor, its size, its location, and the amount that it has spread determines the type of treatment for rhabdomyosarcoma. Clinical group and stage (described in “Description”) determine the type of treatment. Rhabdomyosarcoma that continues to grow during treatment or that comes back once treatment is finished is often resistant to subsequent treatment.

SUGGESTED PROGRAMMATIC ASSESSMENT*

Suggested MER for Evaluation:

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  Clinical examination;

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  Biopsies;

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  Imaging tests;

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  Surgical notes;

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  Pertinent treatment records; and

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  Up-to-date progress notes.

Suggested Listings for Evaluation:

REMARKS

Rhabdomyosarcoma in adults, the criteria in listing 13.04 is met with evidence of regional or distal metastases, or persistent or recurrent following initial antineoplastic therapy.