What you need to know about Rhabdomyosarcoma

Rhabdomyosarcoma (RMS) is a cancerous tumor of the muscles that are attached to the bones. It can occur in almost any part the body. Although these tumors can arise almost anywhere, the most common locations for these tumors to develop are in the structures of the head and neck (nearly 40% of all cases), the male or female genitourinary tract (about 25% of all cases), and the extremities —  arms or legs and trunk (chest and abdomen), (about 20% of all cases).

RMS is the most common soft tissue tumor in children, especially those under 15 years of age. It is also the most common soft tissue sarcomas of adolescents and young adults. It is rare in persons older than 45 years.

Not very much is known about why normal skeletal muscle cells become cancerous. RMS is a very rare cancer. There are only about 350 cases of RMS diagnosed each year in the United States in children under the age of 21 years.

Rhabdomyosarcoma is very uncommon in adults. Adults are more likely to have faster-growing types of RMS and to have them in parts of the body that are harder to treat. Because of this, RMS in adults is often harder to treat effectively.

Symptoms

Symptoms of adult soft tissue sarcoma may not be noticeable until the cancer has gone past its early stages. The first sign is usually a mass or a swollen lump. The mass may or may not be painful.  If the tumor affects muscles or nerves, it may be painful, but this usually is not the case. Other symptoms may vary depending on where the tumor develops. It may cause bloody stool or abdominal pain if it develops in the abdomen area.

Tumors that arise in the legs or arms are usually amongst the most aggressive types of RMS. These tumors may grow from the size of a mosquito bite or a small marble to the size of a baseball or grapefruit in the course of only a few weeks. The tumors are usually hard, but only rarely are they painful unless they start pressing on nearby nerves.

Treatment

Often in young people and children chemotherapy is given first to try and shrink the tumour, before surgery. If all of the tumour is destroyed by chemo, then there may be follow-up radiotherapy.

Surgery: The aim of surgery is to remove the tumour. Sometimes, because of the type of tumour it can often be difficult to remove. It depends on where the rhabdomyosarcoma is as to the details of the operation. If the sarcoma is on the legs or arms, sometimes some of the bone will have to be removed. Either a prosthesis (a metal replacement bone), or a bone graft (bone taken from another part of the body) will be inserted to replace what is missing. This is known as limb-sparing surgery.

Unfortunately because of the location of the tumour, sometimes limb-sparing surgery doesn’t work and a limb might have to be amputated.

Surgical resection with negative margins remains the standard primary treatment. But adequate margins are often difficult to obtain. Consequently, recurrent disease and systemic relapse remain a significant problem.

The role of surgery in the management of patients with RMS is clearly site-specific. Superior outcome has been suggested when initial complete, gross total, or even debulking surgery is performed for patients with unfavorable site tumors.

It is important to remember that surgery by itself is never curative. It is also important to remember that the role of surgery is very dependent on the site of the tumor. Initial complete surgical removal of tumors arising in an extremity or in the pelvis may help improve the chance of cure.

Radiotherapy: This is often given to people with larger rhabdomyosarcomas. It can be given after surgery to ensure that any remaining cancer cells are destroyed. Sometimes it is given before surgery, to try and shrink the tumour and make the surgery easier. Whether you have radiotherapy before or after surgery depends on your individual case.

Radiotherapy  is done  to maximize the chance for cure.

Chemotherapy: Chemotherapy is often given to people with rhabdomyosarcoma to shrink the tumour prior to surgery. This is called neo-adjuvant treatment (chemo before surgery) and usually means that the surgery will be less invasive because the tumour is smaller.

Alternatively, chemo might be given after surgery to kill any remaining cancer cells and stop them from spreading. This is called adjuvant chemotherapy (chemo after surgery).

All patients with RMS require chemotherapy to maximize the chance for cure. Example of drugs are:  Vincristine, Dactinomycin, Cyclophosphamide, Topotecan, Irinotecan, Etoposide, Ifosfamide, Doxorubicin, Carboplatin.

Late Effects of Treatment

Individual chemotherapy agents may have unique toxicities that may not become manifest until many years after the end of therapy, or that may steadily worsen with increased length of follow-up.

Damage from radiation therapy, and late complications from surgery, may not become apparent for many years. The long-term side effects of treatment are:

  1. Infertility(associated especially with the use of alkylating agents such as cyclophosphamide and ifosfamide).
  2. Bladder dysfunction: Although “non-mutilating” conservative surgery and full-dose irradiation has become the treatment of choice for bladder preservation in children with bladder/prostate RMS, approximately half of children with “intact” bladders will have one or more symptoms of bladder dysfunction including dribbling, incontinence, and enuresis.
  3. Radiation damage of head and neck structures: Well described complications of radiation include cataract formation,  asymmetric facial growth as a result of permanently arrested bone development and fibrosis (“scarring”) of surrounding tissues; chronic sinus infections; growth failure due to pituitary damage; and complex and multiple dental abnormalities.
  4. Secondary cancer: Perhaps the most devastating late complication of treatment for any type of cancer, not just RMS, is the development of a second form of cancer. The use of chemotherapy and radiation can cause second cancers to develop. Chemotherapy-associated secondary cancers are most commonly leukemias (typically Acute Myeloid Leukemia [AML]), and may be associated with the use of alkylating agents (cyclophosphamide and ifosfamide), and topoisomerase II inhibitors (etoposide and doxorubicin).
  5. Radiation is also associated with the development of second cancers, most commonly other sarcomas (either in bone or soft tissue). At the doses of radiation that are currently used to treat children with RMS, the risk of secondary sarcomas is approximately 5% at 20 years. Unlike the situation with secondary leukemias, which typically develop within four years of treatment, most cases of secondary sarcomas do not develop until 5+ years after the end of treatment.

Recurrence & Metastasis

Post-relapse survival for the majority of patients with recurrent RMS remains dismal. 95% of recurrences occur within three years of diagnosis.

Metastases develop during the course of the disease or, in about 20% of cases, are present at diagnosis.

Major metastatic sites are the lung, lymph nodes and bone marrow followed by the heart, brain, meninges, pancreas, liver and kidney.

The lungs are involved in at least two-thirds of patients with metastasis.

Source

http://www.cancer.org/cancer/rhabdomyosarcoma/detailedguide/rhabdomyosarcoma-what-is-rhabdomyosarcoma

http://www.nlm.nih.gov/medlineplus/ency/article/001429.htm

http://sarcomahelp.org/rhabdomyosarcoma.html

http://www.sharecare.com/health/soft-tissue-sarcoma-adult/what-symptoms-adult-soft-tissue

https://nowwhat.org.au/about-cancer/cancer-types/soft-tissue-sarcomas/rhabdomyosarcoma/

Soft Tissue – Chapter 22: Rhabdomyosarcoma  by Sharon Weiss & John Goldblum

 

When I made a google search on “Can rhabdomyosarcoma be cured?” or “Effectiveness of treatment of rhadomyosarcoma” these are the pathetic answers I got.

  • Unfortunately, there is no guaranteed cure for rhabdomyosarcoma. However, treatment can be very effective, especially if the cancer is caught early. The most effective treatment usually includes surgical removal of the tumor and some surrounding tissue, followed by another treatment — usually chemotherapy or radiation therapy — to kill any cancer cells that may be left after surgery. http://www.sharecare.com/health/soft-tissue-sarcoma-adult/what-symptoms-adult-soft-tissue
  • When discussing cancer survival statistics, doctors often use a number called the5-year survival rate. The 5-year survival rate refers to the percentage of patients who live at least 5 years after their cancer is diagnosed. If the cancer has spread widely, the 5-year survival rate is generally around 20% to 40%.  http://www.cancer.org/cancer/rhabdomyosarcoma/detailedguide/rhabdomyosarcoma-staging-survival-rates
  • In patients with localised disease, overall 5-year survival rates have improved to more than 80% with the combined use of surgery, radiotherapy and chemotherapy. However, there has been little progress in managing metastatic disease with a 5-year event-free survival rate less than 30%. http://www.patient.co.uk/doctor/Rhabdomyosarcoma

Related posting:  Metastatic Rhabdomyosarcoma: Surgery, radiation and chemotherapy did not cure her.

 

 

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