Rhabdomyosarcoma

Symptoms are based on where the cancer is found and whether or not it has spread to other locations.

This cancer is usually not painful, but it can grow large and can spread to other locations before it is recognized. It can cause bleeding, trouble passing urine and large, growing lumps.

• RMS of the bladder or prostate can make it hard to pass urine and show blood in the urine.
• RMS in the vagina may cause a protruding lump or a smelly, bloody discharge.
• RMS in the paratesticular area causes a swollen, painless lump in the scrotum or groin area.

Your child's doctor will order screening tests to properly diagnose RMS. An ultrasound can confirm tumors. After that, more tests will be done. The below tests can show exactly if and where there are tumors:

• CT scan
• MRI
• Chest X-ray
• Blood tests of liver function
• Bone scan
• Bone marrow tests
• Biopsy

RMS is treated with combination of surgery, chemotherapy and radiation. Chemotherapy drugs are given in the hospital (by IV or orally) to kill cancer cells everywhere. Radiation is a series of x-ray treatments given to kill cancer cells in a specific area. Surgery is intended to remove the entire cancer, part of it, or for diagnosis by getting a biopsy of the lump or an involved lymph node.

Since rhabdomyosarcoma is a rare cancer, patients should aim to find an expert. Ideally, you can find one in a nearby children’s hospital that has specialists in both pediatric oncology, pediatric surgery and pediatric urology. Clinical Trials may be a helpful option for a child with RMS.

Talk with an oncologist or urologic oncologist who has experience with treating this cancer.

Treating Bladder and Prostate RMS Tumors

First, biopsy of the bladder or prostate mass is needed to establish the diagnosis. Surgery has shifted from removal of the organs to a more conservative organ-sparing approach with similar outcomes. A CT scan or MRI will show if cancer has spread to local lymph nodes. In this case open biopsy and removal of these nodes for examination may follow. Second, chemotherapy is used to shrink tumors. There are many helpful chemotherapy drugs for this, some of which include:

• Vincristine
• Dactinomycin
• Cyclophosphamide or ifosfamide

After chemotherapy, assessment of residual cancer will determine if radiation therapy will be needed. Not all residual lumps necessarily contain cancer cells. Second-look surgery and biopsy may be needed to know if residual cancer cells are present. If the bladder is involved, only a portion of the bladder is removed (partial cystectomy) if possible. If the bladder is completely removed, then reconstruction is needed. During the same surgery, part of the intestine is reshaped to store urine. Sometimes a colostomy (bag for stool) and/or a urostomy (bag for urine) are used.

Treating Vaginal RMS

In 80% of vaginal RMS cases, limited surgery and chemotherapy can cure this cancer. After chemotherapy, a biopsy can confirm if the cancer has been treated.

Treating Paratesticular RMS

This is mostly found in very young infants, or in teens. To rule out testis cancer in teens, a blood test should be done.

A CT scan or MRI will show if RMS has spread to the lymph nodes. CT scan is accurate in the diagnosis of lymph node involvement in patients less than 10 years of age. Patients who are older than 10 years, surgery to remove all lymph nodes from the abdomen is necessary (in the side of tumor only) even if their CT scan showed no lymph node involvement. This surgery is called "retroperitoneal lymph node dissection or RPLND."

Surgery, chemotherapy and radiation all cause side effects. It helps to learn what to expect before you start treatment. Talk with your health care team about how to manage side effects first. Some things to think about:

• If part of the bladder is removed, it holds less urine. Some people find they will have to go to the bathroom more often.
• Radiation and surgery to the pelvis can harm nerves to the bladder and prostate. This may lead to incontinence (leaking). The sphincter (a muscle that keeps urine in the bladder) may become weak.
• If part of the vagina is removed, some girls may lose sexual sensation or have other sexual dysfunction.
• If retroperitoneal lymph nodes are removed, a boy may not ejaculate normally, which can lead to fertility problems. A surgeon with experience can help. Some teens opt to “bank” sperm before surgery. This decision should be carefully talked about with the family.

In any case, after-care is of great value. A second-look operation is helpful to check for any remaining problems. Patients will need regular CT scans and biopsies over time.

The goal of treatment and after-care is to learn if the entire cancer is gone.

Are there any long-term risks from chemotherapy and radiation for paratesticular RMS patients?

People who have high doses of chemotherapy or radiation are at risk for other cancers. Risks are higher for lymphoma, leukemia and, sarcoma. This occurs in a small number of patients, usually at least after 10 years of recovery. Long-term follow up care is helpful.

Chemotherapy can cause the testes to be small and sperm count to be low. Testosterone replacement is not often needed and fertility treatment can be needed.

What have studies taught doctors about the treatment of rhabdomyosarcoma?

Scientists have learned that radical surgery to remove organs and tissues is often not needed. The best way to treat RMS is with a combination of surgery, chemotherapy and radiation. This can lower the risks of incontinence and sexual dysfunction.

Are there controversies in rhabdomyosarcoma treatment?

Combination therapy can cure rhabdomyosarcoma, but scientists and oncologists continue to work on the balance between the different types of treatment. Research to learn the best treatment for a better quality of life is still going on.

Can paratesticular rhabdomyosarcoma appear later in life?

Paratesticular rhabdomyosarcoma can show up in adult men. CT scans are the best way to find if retroperitoneal lymph nodes were involved. A large study from Indiana University suggests that adult patients should have their lymph nodes removed, then get chemotherapy.

If rhabdomyosarcoma affects the vagina, will treatment alter sexual function?

If part of the vagina is removed during surgery, the uterus and ovaries are untouched. Often, girls will grow up with normal sexual function.

Questions to ask your Health Care Team