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Health Information - Rhabdomyosarcoma
Health Information - Rhabdomyosarcoma
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  Rhabdomyosarcoma
Rhabdomyosarcoma
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 Clinical Folios: Chest Wall Rhabdomyosarcoma ...
Clinical Folios: Chest Wall Rhabdomyosarcoma...
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Rhabdomyosarcoma
Classification and external resources
ICD-10 C49..M50
ICD-9 171.9
ICD-O: M8900/3-M8920/3
DiseasesDB 11485
MedlinePlus 001429
eMedicine ent/641 ped/2005
MeSH D012208

A rhabdomyosarcoma is a type of cancer, specifically a sarcoma (cancer of connective tissues), in which the cancer cells are thought to arise from skeletal muscle progenitors. It can also be found attached to muscle tissue, wrapped around intestines, or in any anatomic location. Most occur in areas naturally lacking in skeletal muscle, such as the head, neck, and genitourinary tract.

Contents

[edit] Presentation

Its two most common forms are embryonal rhabdomyosarcoma and alveolar rhabdomyosarcoma. In the former, which is more common in younger children, the cancer cells resemble those of a typical 6-to-8-week embryo. In the latter, which is more common in older children and teenagers, they resemble those of a typical 10-to-12-week embryo.

[edit] Epidemiology

Rhabdomyosarcoma is a relatively rare form of cancer.

It is most common in children ages one to five, and is also found in teens aged 15 to 19, although this is more rare. This cancer is also an adult cancer but it is rare.

St. Jude Children's Research Hospital reports that rhabdomyosarcoma is the most common soft tissue sarcoma in children. Soft tissue sarcomas make up about 3% of childhood cancers.

[edit] Diagnosis

When rhabdomyosarcoma is suspected, tests will be run for blood, muscle, and marrow.

Diagnosis of rhabdomyosarcoma depends on recognition of differentiation toward skeletal muscle cells. The protein myo D1 is a protein normally found in developing skeletal muscle cells which disappears after the muscle matures and becomes innervated by a nerve. Thus, myo D1 is not found in normal skeletal muscle and serves as a useful immunohistochemical marker of rhabdomyosarcoma. Early manifestation can be misdiagnosed as a pseudotumor that is non responsive to steroid treatment.

[edit] Treatment

Treatment for rhabdomyosarcoma consists of chemotherapy, radiation therapy and sometimes surgery. Surgery to remove the tumor is often difficult or impossible because the tumor is usually embedded deep within the tissue, leaving it difficult to reach. If a tumor presents itself in the extremities, amputation is often necessary to improve chances of survival.

If there is no evidence of metastasis, surgery combined with chemotherapy and radiation offer the best prognosis. Patients whose tumors have metastasized usually have a poor chance for long-term survival.

St Jude's Children's Research Hospital reports that more than 70% of children diagnosed with localized rhabdomyosarcoma have long-term survival.

[edit] Tungsten Alloy and RMS

A study has shown a link between RMS and Tungsten alloy embedded in muscle (e.g. shrapnel). [1] However it should be noted that the tungsten alloy contained 6% Nickel and 3% Cobalt, which are known carcinogens. A synergistic effect of alloying tungsten with Ni and Co is suggested (i.e. increasing the toxicity relative to either of the pure metals).

[edit] See also

[edit] References

  1. ^ http://www.afrri.usuhs.mil/www/outreach/pdf/tungsten_cancer.pdf

[edit] External links





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