RHABDOMYOSARCOMA

ALTERNATE NAMES

Alveolar Rhabdomyosarcoma; Embryonal Rhabdomyosarcoma; Sarcoma Botryoides; Soft Tissue Sarcoma

DESCRIPTION

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: I, II, III, and 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 1, 2, and 3 may have evidence of spread to lymph nodes but they do not have evidence of spread to distant sites. Stage 4 has spread to lymph nodes and distant sites. Group IV, Stage 4 is the most severe. It has the lowest survival rate. Rhabdomyosarcoma is much more common in children than adults.

DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM CODING

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 biopsy of tumor; X-rays; CT scan of tumor site and chest (to look for spread of tumor); PET scan; bone scan; bone marrow biopsy; MRI of tumor site; blood and urine tests; and spinal tap (lumbar puncture) to check the cerebrospinal fluid.

ICD-9: 171.9

ONSET AND PROGRESSION

Rhabdomyosarcoma usually occurs in two distinct groups: children age five and under, and adolescents 14-20 years of age. Generally, children with Stage 4 rhabdomyosarcoma have a 5-year survival rate of 20 to 25%. Survival rates for Stages 1, 2, and 3 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.

TREATMENT

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: The adjudicator needs medical evidence from treating sources and hospitals. It should include clinical examination, biopsies, imaging tests, surgical procedures, pertinent treatment records, and up-to-date progress notes.

Suggested Listings for Evaluation:

DETERMINATION

LISTING

REMARKS

Meets Listing

13.04

113.03

Rhabdomyosarcoma of any grade in children meets 113.03; in adults, 13.04 is met with evidence of regional or distal metastases, or persistent or recurrent following initial antineoplastic therapy.

Medical Equals