Rhabdomyosarcoma

Rhabdomyosarcoma is a cancerous (malignant) tumor of the muscles that are attached to the bones.

It can occur in many places in the body. The most common sites are the structures of the head and neck,
the urogenital tract, and the arms or legs.

Rhabdomyosarcoma is the most common soft tissue tumor in children.

Causes

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 an increased risk, and some families have a gene mutation
that elevates risk. However, the great majority of children with rhabdomyosarcoma do not have any
known risk factors.

Symptoms

The most common symptom is a mass that may or may not be painful.

Other 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 around the eyes may cause bulging of the eye, problems with vision, swelling around the
eye, or pain.
 Tumors in the ears, may cause pain, hearing loss, or swelling.
 Tumors in the vagina may be visibly protruding from the opening of the vagina.
 Bladder and vaginal tumors may cause lead to trouble starting to urinate or having a bowel
movement, or poor control of urine.
 Muscle tumors may lead to a painful lump and are often thought to be an injury.

Exams and Tests

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.

A complete physical exam should be done. Tests done to diagnose this condition may include:

 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
  Spinal tap (lumbar puncture)

Treatment

The precise treatment depends on the site and type of rhabdomyosarcoma. Because of its rarity, this
tumor is best treated at a center with experience treating many patients with rhabdomyosarcoma.

Either radiation or chemotherapy, or both, will be used before or after surgery. In general, surgery and
radiation therapy are used to treat the primary site of the tumor. 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:

 Dacarbazine
 Doxorubicin
 Epirubicin
 Gemcitabine
 Ifosfamide

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.

Outlook (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.

Possible Complications

 Complications from chemotherapy

 Location in which surgery is not possible
 Metastasis

When to Contact a Medical Professional

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

Alternative Names

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

Rhabdomyosarcoma, pleomorphic - Hand
Rhabdomyosarcoma, alveolar – Thigh

ORBIT
Rhabdomyosarcoma is a fast-growing, highly malignant tumor which accounts for over half of the
soft tissue sarcomas in children. Less frequently, other soft tissue sarcomas are found in children:
fibrosarcoma, mesenchymoma, synovial sarcoma, and liposarcoma.
Rhabdomyosarcoma often causes a noticeable lump on a child's body. If the tumor is located
internally, the symptoms depend on its location. For example, tumors in the nasal passage may put
pressure on the eustachian tubes; a bladder tumor can cause trouble urinating; an orbital tumor
may cause the eye to protrude.
About Rhabdomyosarcoma
Rhabdomyosarcoma tumors arise from a cell called a "rhabdomyoblast", which is a primitive muscle
cell. Instead of differentiating into striated muscle cells, the rhabdomyoblasts grow out of control.
Since this type of muscle is located throughout the body, the tumors can appear at numerous
locations. The four major sites in which rhabdomyosarcoma is found are:
  head and neck; around the eyes -- 35-40%
 genitourinary tract -- 20%
 extremities -- 15-20%
 trunk (chest and lungs) -- 10-15%

Depending on the "histology" of the cells (how they look under a microscope), the tumors are
classified as one of the following:
Embryonal rhabdomyosarcoma. Most common type, usually found in children under 15 and in the
head and neck region and genitourinary tract.
Botryoid type. A variant of the embryonal type; the tumor arises as a grape-like lesion in mucosal-
lined hollow organs such as the vagina and urinary bladder.
Alveolar type. A more aggressive tumor which usually involves the muscles of the extremities or
trunk.
Pleomorphic type. Usually seen in adults and arises in muscles of the extremities.
Embryonal rhabdomyosarcoma is considered the most treatable form of the disease. The prognosis
is also affected by the location of the primary tumor. Orbital and genitourinary track
rhabdomyosarcomas have a better prognosis than do tumors which originate in the head and neck,
extremity, pelvic, and trunk locations.
Prognosis also depends on the stage of the tumor. The Intergroup Rhabdomyosarcoma Study Group
has defined a set of guidelines, which assign the tumor to groups 1-4 depending on the extent of the
disease. For more information, see the PDQ on the staging of rhabdomyosarcoma.
Treatment
Rhabdomyosarcoma is treated by a combination of surgery, chemotherapy, and radiation.
Surgery. Resection (removal) of the primary tumor. If necessary after chemotherapy or radiation
has shrunk the tumor.
Chemotherapy. The following chemotherapy agents are commonly used: vincristine,
cyclophosphamide, dactinomycin, adriamycin, ifosfamide, VP-16.
Radiation. External beam radiation is used in some cases of rhabdomyosarcoma.
Rhabdomyosarcoma clinical trials, NCI search form
 Statistics
 Accounts for 5-8% of childhood cancers.
 70% of all rhabdomyosarcoma cases diagnosed in the first ten years of life.
 Usually affects children the ages of 2 to 6 and 15 to 19.
 The peak incidence in 1-5 age group.
 Overall, 50% of the children diagnosed with rhabdomyosarcoma survive 5 years.
Ped-Onc Resources for Rhabdomyosarcoma
The following ped-onc resource lists have appropriate sections for parents of children with
rhabdomyosarcoma:
 childhood cancer e-mail lists - the ACOR rhabdomyosarcoma list (Rhabdo-kids) and the general
childhood cancer list, ped-onc, are both appropriate
 support organizations
 books and printed materials - especially the book Childhood Cancer, A Parents's Guide to Solid
Tumor Cancers, by Honna Janes-Hodder and Nancy Keene.
 young people with rhabdomyosarcoma - personal home pages