Rhabdomyosarcoma

 ICD9        = 171.9|

}} A rhabdomyosarcoma is a type of cancer, specifically a sarcoma(cancer of connective tissues), in which the cancer cells arise from skeletal muscle. It can also be found attached to muscle tissue, wrapped around intestines, or anywhere, to include the neck area. It is most common in children ages one to five, and teens aged 15 to 19, although quite rare in the latter.

It can be a cardiac manifestation of tuberous sclerosis.

Diagnosis

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

Diagnosis of rhabdomyosarcoma depends on recognition of differentiationtoward 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 histochemical marker of rhabdomyosarcoma.

Treatment

Treatment for rhabdomyosarcoma consists of chemotherapy, radiation therapyand 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 survival. If there is no evidence of metastasis, surgery combined with chemotherapy and radiation offer the best prognosis. Patients whose tumors have metastisized usually have a poor chance for long-term survival. In patients who began treatment before metastasis, the prognosis is better, although the disease is generally incurable because the tumors that cannot be surgically removed tend to spread.

External links

• Ped-Onc Resource Center
• eMedicineped/2005
• MedlinePlus Encylopedia001429

This article is licensed under the GNU Free Documentation License.
It uses material from the http://en.wikipedia.org/wiki/Rhabdomyosarcoma Wikipedia article Rhabdomyosarcoma.