Bay Biosciences provides high quality Rhabdomyosarcoma tumor tissue samples FFPE blocks from unique pediatric patients for research, development and discovery. Detailed clinical data and pathology annotations including patient’s corresponding diagnostic test values associated with the Rhabdomyosarcoma tumor tissue samples are provided to a valued pharmaceutical customer for In-vitro research and drug discovery.
Rhabdomyosarcoma (RMS) Tumors Overview
Rhabdomyosarcoma (RMS) is a rare type of highly aggressive cancerous tumor that forms in soft tissue such as muscles, specifically skeletal (voluntary) muscle tissue or sometimes in hollow organs such as the bladder or uterus. RMS can occur at any age, but it most often affects children. Rhabdomyosarcoma (RMS) can affect the head, neck, bladder, vagina, arms, legs, trunk, or just about any body part. Cells from rhabdomyosarcomas are often fast growing and can spread (metastasize) to other parts of the body. RMS is a very rare cancer. There are only about 350 cases of RMS diagnosed each year in the United States in children under the age of 21 years.
Types of Rhabdomyosarcoma (RMS) Tumors
Following are the two main types of Rhabdomyosarcoma (RMS) are:
- Embryonal Rhabdomyosarcoma (ERMS): ERMS usually affects children in their first 5 years of life, but it can occur at older ages as well. ERMS tends to occur in the head and neck area, bladder, vagina, or in or around the prostate and testicles.
Two subtypes of ERMS: - – Botryoid Rhabdomyosarcoma
- – Spindle Cell Rhabdomyosarcomas, tend to have a better prognosis (outlook) than the more common conventional form of ERMS.
- Alveolar Rhabdomyosarcoma (ARMS): ARMS typically affects all age groups equally. It makes up a larger portion of RMS in older children, teens, and adults than in younger children (because ERMS is less common at older ages). ARMS most often occurs in large muscles of the trunk, arms, and legs. ARMS tends to grow faster than ERMS, and it usually requires more intense treatment. However, in some cases of ARMS, the cancer cells lack certain gene changes, which makes these cancers act more like ERMS.
Causes of Rhabdomyosarcoma (RMS) Tumors
The cause of most cases of Rhabdomyosarcoma (RMS) are unknown, and there are very few known risk factors for this type of cancer. But researchers are learning how normal cells become cancerous because of certain changes in their DNA.
Some patients with Rhabdomyosarcoma (RMS) have inherited gene changes from a parent that put them at higher risk. For example, people with Li-Fraumeni syndrome have changes in the p53 tumor-suppressor gene, which cause it to make a defective p53 protein. The p53 protein normally causes cells with DNA damage to either pause and repair that damage or, if repair is not possible, to self-destruct. When p53 is not working, cells with DNA damage keep dividing, which can lead to further defects in other genes, and eventually causing cancer.
Gene changes in Alveolar Rhabdomyosarcoma (ARMS)
Certain genes in a cell can be turned on when bits of DNA are switched from one chromosome to another. This type of change, called a translocation, can happen when a cell is dividing into 2 new cells. This seems to be the cause of most cases of alveolar rhabdomyosarcoma (ARMS). In these cancers, a small piece of chromosome 2 (or, less often, chromosome 1) often ends up on chromosome 13. This moves a gene called PAX3 (or PAX7 if it’s chromosome 1) right next to a gene called FOXO1, creating a PAX/FOX01 fusion gene. The PAX genes play an important role in cell growth while an embryo’s muscle tissue is being formed, but these genes usually shut down once they’re no longer needed. Moving one of them next to the FOXO1 gene keeps the PAX gene active, which seems to be what leads to the tumor forming.
Other translocations or other types of gene changes are also sometimes seen in ARMS. In fact, about 1 out of 4 cancers that doctors would usually classify as ARMS have been found not to have the PAX/FOXO1 fusion gene typically seen in ARMS. Researchers have found that these cancers act more like embryonal rhabdomyosarcoma (ERMS), which generally requires less intensive treatment than ARMS.
Gene changes in Embryonal Rhabdomyosarcoma (ERMS)
Research suggests that embryonal rhabdomyosarcoma (ERMS) develops in a different way. Cells of this tumor typically have lost a small piece of chromosome 11 that came from a person’s mother, and it has been replaced by a second copy of that part of the chromosome from their father. This seems to make the IGF2 gene on chromosome 11 overactive. The IGF2 gene codes for a protein that can make these tumor cells grow. Other gene changes are probably important in these tumors as well.
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