Advancement in the use of radiation therapy includes using three-dimensional conformal radiation therapy (3D-CRT) to create a three-dimensional image of tumor so providers can determine the dose of radiation per patient while limiting radiation exposure to normal tissues. Techniques such as multi-field optimization (MFP) allows for more precise distribution of proton beams. Treatment Plan
The first line of treatment for most people is surgery or resection of the tumor(s). The surgery involves removing the primary tumor and any tissue that may be infected with cancer cells. Resection of the tumor tends to be more favorable in cancers that have not yet metastisized. In the case that the tumor cannot be removed, the cancer can be treated with a combination of radiation and chemotherapy instead. In the majority of people, the surgery cannot remove all traces of the cancer and chemotherapy/radiation will be required as further treatment to eradicate any remaining cancer. Chemotherapy is given in the US as a medication regimen abbreviated VAC, consisting of Vincristine, Actinomycin D, and Cyclophosphamide. The chemotherapy works to kill all the remaining cancer cells and to stop future growth of any possible cancerous cells. Safety concerns regarding the long term effects of chemotherapy remains a concern. Side effects of the VAC regimen include nausea, vomiting, liver damage, and immune system suppression. The length and dosing of the chemotherapy is oftentimes on a case by case basis based off the persons stage of cancer, site of tumor and age.
In either case, "fusion-negative" alveolar rhabdomyosarcoma have similar clinical presentation and outcome as embryonal rhabdomyosarcoma, thus risk stratification, prognosis, and treatment intensity of rhabdomyosarcoma are now determined by fusion-status instead of histological classification. Imaging
After a physical exam, formal diagnosis of RMS in adult patients requires a computed tomography (CT) scan, which can assess the areas affected and to delineate the tumor. In children, physicians may opt for magnetic resonance imaging (MRI) to limit radiation exposure in younger populations. In the majority of individuals diagnosed with rhabdomyosarcoma, more than half are diagnosed before the age of 10. Prognosis
The prognosis for rhabdomyosarcoma has improved greatly in recent decades, with over 70% of people surviving for five years after diagnosis. The combined use of radiotherapy and surgery has significantly reduced the mortality rates compared to patients who did not undergo any radiotherpay or chemotherapy treatments. Embryonal rhabdomyosarcoma is generally associated with better prognosis than alveolar rhabdomyosarcoma, with a 5-year survival prognosis of 82% and 53%, respectively. This may be due to the more aggressive and metastatic nature of ARMS that can be attributed to its PAX3–FOXO1 or PAX7–FOXO1 gene fusions. Nevertheless, some embryonal rhabdomyosarcoma patients with a rare Leu122Arg mutation in MYOD1 gene have a very poor outcome. In two different studies, none of the subjects with the MYOD1 mutation survived. Tumors due to this mutation commonly manifest in the head and neck area, causing the mutated protein to behave like an oncogene.There have not been many studies linking the genetic profile and clinical outcome of ERMS.