Treatments by location of the tumour

The healthcare team will plan treatments based on where the rhabdomyosarcoma (RMS) started in the body.

The areas of the head and neck where RMS can develop include:

• around the eye (called orbital RMS)
• in the tissue surrounding the brain and spinal cord, or meninges (called parameningeal RMS)
• in the areas that are not close to the meninges (called non-parameningeal RMS)

Orbital RMS is treated with chemotherapy and radiation therapy. It often responds well to these treatments.

Parameningeal RMS includes RMS that starts in the nasal passages, paranasal sinuses, middle ear and back of the upper throat. Tumours in these areas often can't be removed with surgery because of their location. They are often treated with radiation therapy and chemotherapy.

Non-parameningeal RMS may be treated using surgery if the tumour is close to the skin. The surgeon may also remove some lymph nodes from the same side of the neck as the tumour. Chemotherapy is usually given after surgery. Sometimes radiation therapy is given with chemotherapy.

The areas of the trunk where RMS can develop include:

• chest, abdomen or pelvis
• diaphragm
• around the anus (called perianal RMS)
• in the area between the anus and the scrotum or vagina (called perineal RMS)
• gallbladder and bile ducts

Tumours in the chest or abdomen are usually removed with surgery. Chemotherapy can be given both before and after surgery. If the tumour can't be completely removed by surgery, chemotherapy is usually given first. Then surgery, radiation therapy or both are used to treat the tumour, followed by more chemotherapy.

Tumours in the pelvis are often difficult to remove with surgery. If it is possible to remove the tumour, surgery may be done. If the tumour is large, chemotherapy, sometimes with radiation therapy, may be given first to shrink the tumour before surgery. Some tumours in the pelvis may be treated with radiation therapy after a surgical biopsy, in addition to chemotherapy.

Tumours in the diaphragm are treated with chemotherapy and radiation therapy. Surgery may or may not be done after these treatments.

Perianal RMS and perineal RMS are usually treated with surgery to remove as much of the tumour as possible, as well as nearby lymph nodes. Surgery is followed by chemotherapy.

Tumours in the gallbladder and bile ducts often can't be completely removed with surgery. Surgery is used to remove as much of the tumour as possible. Chemotherapy and radiation therapy are given after surgery.

The areas of the genitourinary system where RMS can develop include:

• around the testicles (called paratesticular RMS)
• bladder
• prostate
• vulva
• vagina
• uterus
• ovaries

Paratesticular RMS is treated with surgery to remove the testicle and spermatic cord. If the child is at least 10 years old, removing nearby lymph nodes (called a retroperitoneal lymph node dissection, or RPLND) is usually recommended. After surgery, radiation therapy may be given to the area where the tumour was removed and nearby lymph nodes if there are cancer cells in them.

Tumours in the bladder or prostate are usually removed with surgery. Chemotherapy, sometimes with radiation therapy, is given before surgery to shrink the tumour so that less of the bladder or prostate needs to be removed and they will work as normally as possible. If the tumour is in the top of the bladder, surgery can be done to remove the tumour before treating the area with chemotherapy or radiation therapy.

Tumours in the vulva, vagina, uterus, ovaries or cervix usually respond well to chemotherapy. Chemotherapy is usually given first followed by radiation therapy or surgery to completely remove the tumour, if possible. For RMS in the uterus, radiation therapy may be used with chemotherapy.

RMS can happen in the following extremities:

• arm
• leg
• hand
• foot

RMS in the arm or leg can be removed by surgery if it is possible to remove the tumour without affecting how the limb works. Nearby lymph nodes may also be removed to check if cancer has spread there. Chemotherapy, sometimes with radiation therapy, is often given after surgery.

If surgery is not possible, chemotherapy and radiation therapy may be used first to try to shrink the tumour so that it may then be removed with surgery. In rare cases, doctors may need to remove (amputate) the affected limb if they can't remove the tumour with surgery and it does not respond to chemotherapy and radiation therapy.

RMS in the hand or foot is often treated with chemotherapy and radiation therapy rather than surgery. This is because surgery can affect how well the hand or foot works.