A rhabdomyosarcoma, commonly referred to as RMS, 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 (similar to stem cells). It can also be found attached to muscle tissue, wrapped around intestines, or in any anatomic location. It mostly occurs in areas naturally lacking in skeletal muscle, such as the head, neck, and genitourinary tract.
RMS is relatively rare but when discovered it is most commonly found in children between 1 and 5 years old. It is the most common soft tissue sarcoma in children. There are approximately 250 cases per year, more slightly in males than females.
Prognosis for most of those diagnosed with RMS has improved significantly in the last 30 years. Overall survival rates have improved from 25% to more than 70% in recent reports. The primary site of disease, the extent of disease and the subtype influence the prognosis. Favorable primary sites include the eyes, the head and neck region (except the areas near the lining of the nervous system), the vagina and the area near the testis. The extent of the disease, particularly after surgery, is also important. Those who have surgery that completely or almost completely removes all tumors have a better outlook than those who have significant disease remaining after surgery.
Children who present with metastatic disease at diagnosis (approximately 20% of cases) fare less well, but those with limited metastatic sites (two or fewer) and favorable histology can have survival rates approaching 40%. With regard to histology, embryonal rhabdomyosarcoma has a more favorable prognosis than the alveolar subtype.
The treatment of rhabdomyosarcoma (RMS) has come a long way in the past few decades, largely due to the work of the Intergroup Rhabdomyosarcoma Study Group (now known as the Soft Tissue Sarcoma Committee of the Children’s Oncology Group). However, more work needs to be done. Research on RMS is being done at many medical centers, university hospitals, and other institutions around the world.
Treatment for rhabdomyosarcoma consists of chemotherapy, radiation therapy and sometimes surgery. Surgery to remove the tumor may be difficult or impossible depending on the location of the tumor. If there is no evidence of metastasis, surgery combined with chemotherapy and radiation currently offers the best prognosis. Patients whose tumors have not metastasized usually have a good chance for long-term survival, depending on the subtype of the tumor. 87% of children are cured in the 1st protocol. More than 70% of children diagnosed with localized rhabdomyosarcoma have long-term survival.
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