Rhabdomyosarcoma overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
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 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.
Rhabdomyosarcoma is a relatively rare form of cancer. 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.
Classification
Rhabdomyosarcoma may be classified according to the "International classification of rhabdomyosarcoma" into six subtypes Alveolar rhabdomyosarcoma, embryonal rhabdomyosarcoma, botryoid rhabdomyosarcoma, spindle cell rhabdomyosarcoma, and undifferentiated rhabdomyosarcoma.
Pathophysiology
Rhabdomyosarcoma arises from the skeletal muscle cells. Development of rhabdomyosarcoma is result of specific genetic mutations. Genes involved in the pathogenesis of alveolar rhabdomyosarcoma include t(2;13) and t(1;13) chromosomal translocations. The microscopic pathology of rhabdomyosarcoma depends on the histological subtype.
Causes
There are no established causes for rhabdomyosarcoma.
Differential Diagnosis
Rhabdomyosarcoma of orbit must be differentiated from other causes of orbital masses such as orbital pseudotumor, orbital tumors, orbital abscess, and vascular lesions.
Epidemiology and Demographics
Rhabdomyosarcoma is a relatively rare form of cancer. It is most common soft tissue sarcoma in children. The overall incidence of rhabdomyosarcoma in children is approximately 0.45 cases per 100,000 children. Rhabdomyosarcoma tends to affect individuals <45 Years.
Risk Factors
The common risk factors in the development of rhabdomyosarcoma are Beckwith-Wiedemann syndrome, Li-Fraumeni syndrome, Costello syndrome, and Noonan syndrome.
Screening
Screening for rhabdomyosarcoma is not recommended.