Definitions You Should Know

Being diagnosed with Clear Cell Sarcoma can be of many things overwhelming. You will have terms thrown at you and read articles that can be difficult to understand. Knowledge of your disease can help you alleviate some stress along the way. We have compiled a list of some of the terms that you may not understand as you enter this new time in your life and are in hopes that this helps you to some extent.


  • Clear Cell Sarcoma  Clear cell sarcoma (CCS) of soft tissue, not to be confused with clear cell sarcoma of the kidney, is a rare type of cancer primarily affecting teens and young adults. CCS tumors are most commonly found in the limbs, feet and hands. They also develop in the gastrointestinal tract and throughout the torso and have also been found in the genitals and head. Clear cell sarcoma is a translocation-associated sarcoma, typically EWSR1/ATF1.


  • Mutation  the changing of the structure of a gene, resulting in a variant form, caused by the alteration of single base units in DNA, or the deletion, insertion, or rearrangement of larger sections of genes or chromosomes. Mutations may be transmitted to subsequent generations if they occur in the DNA of germ cells (eggs or sperm).


  • Translocation  In chromosomal translocations, parts of two chromosomes are swapped. This can result in an abnormal fusion of genes. The most common types of CCS harbor an EWSR1/ATF1 or a EWSR1/CREB1 translocation. There are some cases of CCS where the translocation is completely different.


  • Cell line  A cell line refers to cells that are isolated from a patient’s tumor which will keep dividing and growing over time. Cancer cell lines are used in research to study the biology of a particular cancer and to test treatments that will affect the growth or survival of those cells. The premise of those studies is that the effective treatment will behave similarly on the cancer in the patient.


  • Xenograft  a tissue graft or organ transplant from a donor of a different species from the recipient.


  • PDX Mouse Model  Tumor tissue that has been taken from a patient and implanted into mice for research purposes. PDX stands for patient derived xenograft. Cancer drugs and other types of treatment may be tested on xenografts to see how well they work before they are given to the patient. PDX mouse models may be used to determine the best treatment plan for a patient. They are also being used in the development of new cancer drugs.


  • Repurposing Drugs  Discovering new uses for already approved drugs in an effort to speed up treatment in diseases with an unmet need, such as rare cancer.


  • Informed Consent  permission granted with the knowledge of the possible consequences, typically that which is given by a patient to a doctor for treatment or participation in a study with full knowledge of the possible risks and benefits.


  • Immunotherapy  Immunotherapy or biological therapy is a type of treatment that helps your immune system fight cancer. The immune system normally helps your body fight infections and other diseases. The immune system made up of white blood cells and organs and tissues of the lymph system. Scientists have found that the immune system can recognize the difference between healthy cells and cancer cells, and with immunotherapy work to eliminate the cancer cells. By itself, the immune system is not always good at destroying cancer cells.


  • PET Scan  Positron emission tomography (PET) scan, is an imaging test that helps reveal how your tissues and organs are functioning. A PET scan uses a radioactive drug (tracer) which accumulates in areas of higher chemical activity. This scan can sometimes detect disease before it shows up on other imaging tests. Cancer cells have a higher metabolic rate than noncancerous cells. Because of this high level of chemical activity, cancer cells show up as bright spots on PET scans. For this reason, PET scans are useful both for detecting cancer and for seeing if it has spread, seeing if a treatment is working and checking for a recurrence. However, these scans should be read carefully by your doctor, as it’s possible for noncancerous conditions to look like cancer on a scan. It’s also common for solid tumors to fail to appear on PET scans.


  • CAT Scan (CT Scan)  Computerized Axial Tomography (CAT) is an X-ray image made using a form of tomography in which a computer controls the motion of the X-ray source and detectors, processes the data, and produces the image. 


  • MRI  Magnetic resonance imaging (MRI) is a technique used to form pictures of the organs and tissues using strong magnets fields. MRI does not involve X-rays or ionizing radiation, which distinguishes it from CT and PET scans.