Intergroup Rhabdomyosarcoma Study Group

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The Intergroup Rhabdomyosarcoma Study Group (IRS) was a U.S. and Canadian clinical trial cooperative group created with the mission of studying childhood cancers. In 2000, IRS merged with several other pediatric cooperative groups to form the Children's Oncology Group (COG). This merger has seen its fair share of problems, especially with regard to integrating the various databases associated with each individual cooperative group. One such initiative to consolidate these databases involves caBIG, or cancer BIG, which is guided and supported by the National Cancer Institute (NCI) in Bethesda, Maryland. They published a report on cancer treatment protocols published by the National Institute of Health in 2001, [1] and a separate report in 2011 on improving outcomes for patients with low-risk embryonal rhabdomyosarcoma. [2]

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Quality Assurance Review Center

The Intergroup Rhabdomyosarcoma Study Group used to have all of its protocol driven cases reviewed at the Quality Assurance Review Center (QARC). As formerly mandated by the National Cancer Institute (NCI), every radiotherapy department participating in an IRS study used to have to submit their data to QARC for review. QARC is located in Providence, Rhode Island, and reviews thousands of cases per year. The center was founded in 1977 as a not-for-profit healthcare organization designed initially to provide quality assurance for CALGB studies. Radiotherapy data from around one-thousand hospitals in both the United States and abroad is reviewed and archived at QARC.

Another center for quality assurance is the Radiological Physics Center (RPC) in Houston, Texas. The primary responsibility of the RPC is to assure the NCI and its former cooperative group IRS that all participating institutions were following the guidelines set-forth for the physics-related aspects of radiotherapy. Established in 1968, the RPC has consistently received funding from the NCI in order to perform the aforementioned mission.

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A rhabdomyoblast is a cell type which is found in some rhabdomyosarcomas. When found histologically, a rhabdomyoblast aids the diagnosis of embryonal, alveolar, spindle cell/sclerosing, and pleomorphic rhabdomyosarcomas; however, in a tumor, expression of the rhabdomyoblast phenotype is not the only factor in diagnosing a rhabdomyosarcoma. Mesenchymal malignancies can exhibit this phenotype as well. Immunohistochemistry techniques allow for the sensitive detection of desmin, vimentin, muscle specific actin, and MyoD1. Similarly the rhabdomyoblast phenotype can be detected morphologically. Rhabdomyoblasts are early stage mesenchymal cells, having the potential to differentiate into a wide range of skeletal cells. Each stage of differentiation exhibits unique and distinguishable histological characteristics. In its initial from, stellate cells with amphiphilic cytoplasm and ovular central nuclei are observed. Commonly referred to as rhabdoid features, the maturing rhabdomyoblast will likely exhibit low levels of eosinophilic cytoplasm in proximal distances to the nucleus. As maturation and differentiation progress, the cell's cytoplasmic levels of white blood cells increase; additionally, elongated shapes, commonly depicted as “tadpole”, “strap” and "spider cells", are observed. In the concluding phase of differentiation, the white blood cell rich cytoplasm appears bright and exhibits cross-striation. The highly regulated organization of actin and myosin microfilaments in contractile proteins results in this appearance.

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References

  1. Raney, R. Beverly; Maurer, Harold M.; Anderson, James R.; Andrassy, Richard J.; Donaldson, Sarah S.; Qualman, Stephen J.; Wharam, Moody D.; Wiener, Eugene S.; Crist, William M. (March 2001). "The Intergroup Rhabdomyosarcoma Study Group (IRSG): Major Lessons From the IRS-I Through IRS-IV Studies as Background for the Current IRS-V Treatment Protocols". Sarcoma. 5 (1): 9–15. doi:10.1080/13577140120048890. ISSN   1357-714X. PMC   2395450 . PMID   18521303.
  2. Beverly Raney, R.; Walterhouse, David O.; Meza, Jane L.; Andrassy, Richard J.; Breneman, John C.; Crist, William M.; Maurer, Harold M.; Meyer, William H.; Parham, David M. (2011-04-01). "Results of the Intergroup Rhabdomyosarcoma Study Group D9602 Protocol, Using Vincristine and Dactinomycin With or Without Cyclophosphamide and Radiation Therapy, for Newly Diagnosed Patients With Low-Risk Embryonal Rhabdomyosarcoma: A Report From the Soft Tissue Sarcoma Committee of the Children's Oncology Group". Journal of Clinical Oncology. 29 (10): 1312–1318. doi:10.1200/JCO.2010.30.4469. ISSN   0732-183X. PMC   3083999 . PMID   21357783.