Clear cell sarcomacancer arising from bones and/or soft tissue of the kidneya pair of bean-shaped organs in the abdomen that are responsible for filtering excess water and waste products from the blood and converting them into urine to be removed from the body (CCSK), also known as renal clear cell sarcoma, is a is a rare type of cancera disease where abnormal cells split without control and spread to other nearby body tissue and/or organs that develops from epithelial and spindle cellsthe basic structural and functional unit of all living things. It is one of the most common renal tumours in childhood after Wilms’ tumoura tissue mass that forms from groups of unhealthy cells, and is often misdiagnosed.
CCSK is often confused with clear cell sarcoma of soft tissuetissue/the material that joins, holds up or surrounds inside body parts such as fat, muscle, ligaments and lining around joints (CCSST), a rare malignancy of soft tissuea group of cells that work together to perform a function that primarily affects young adults. For more information on CCSST, please refer to the Rare Cancers Australia Clear Cell Sarcoma (Soft tissue) page.
CSSK can be aggressive, may metastasise, and may not have as good of a prognosisto predict how a disease/condition may progress and what the outcome might be as other types of childhood cancers, such as Wilms’ tumour. When metastasiswhen the cancer has spread to other parts of the body, also known as mets occurs, CSSK will often spread to the bone, lung, brain and liver, however it can also spread to other places.
CCSK is slightly more common in males, and tends to be diagnosed around the age of three. However, anyone can develop this disease.
Treatment
If a CCSK is detected, it will be staged and graded based on size, metastasis, and how the cancer cells look under the microscope. Stagingthe process of determining how big the cancer is, where it started and if it has spread to other areas and grading helps your doctors determine the best treatment for you.
Cancers can be staged using the TNM staging system:
- T (tumour) indicates the size and depth of the tumour.
- N (nodea small lump or mass of tissue in your body) indicates whether the cancer has spread to nearby lymph nodessmall bean-shaped structures that filters harmful substances from lymph fluid.
- M (metastasis) indicates whether the cancer has spread to other parts of the body.
This system can also be used in combination with a numerical value, from stage 0-IV:
- Stage 0: this stage describes cancer cells in the place of origin (or ‘in situ’) that have not spread to nearby tissue.
- Stage I: cancer cells have begun to spread to nearby tissue. It is not deeply embedded into nearby tissue and had not spread to lymph nodes. This stage is also known as early-stage cancer.
- Stage II: cancer cells have grown deeper into nearby tissue. Lymph nodes may or may not be affected. This is also known as localisedaffecting only one area of body cancer.
- Stage III: the cancer has become larger and has grown deeper into nearby tissue. Lymph nodes are generally affected at this stage. This is also known as localised cancer.
- Stage IV: the cancer has spread to other tissues and organs in the body. This is also known as advancedat a late stage, far along or metastatic cancer.
Cancers can also be graded based on the rate of growth and how likely they are to spread:
- Gradea description of how abnormal cancer cells and tissue look under a microscope when compared to healthy cells I: cancer cells present as slightly abnormal and are usually slow growing. This is also known as a low-grade tumour.
- Grade II: cancer cells present as abnormal and grow faster than grade-I tumours. This is also known as an intermediate-grade tumour.
- Grade III: cancer cells present as very abnormal and grow quickly. This is also known as a high-grade tumour.
Once your tumour has been staged and graded, your doctor may recommend genetic testinga procedure that analyses DNA to identify changes in genes, chromosomes and proteins, which can be used to analyse tumour DNA to help determine which treatment has the greatest chance of success, which analyses your tumour DNA and can help determine which treatment has the greatest chance of success. They will then discuss the most appropriate treatment option for you.
Treatment is dependent on several factors, including location, age, stage of disease and overall health.
Treatment options for CSSK may include:
- Surgerytreatment involving removal of cancerous tissue and/or tumours and a margin of healthy tissue around it to reduce recurrence, potentially including:
- Lymphadenectomysurgical removal of lymph node(s).
- Nephrectomycomplete or partial removal of affected kidney(s).
- Radiation therapya treatment that uses controlled doses of radiation to damage or kill cancer cells.
- Chemotherapya cancer treatment that uses drugs to kill or slow the growth of cancer cells, while minimising damage to healthy cells (may not be recommended for all patients). \Clinical trialsresearch studies performed to test new treatments, tests or procedures and evaluate their effectiveness on various diseases.
- Palliative carea variety of practices and exercises used to provide pain relief and improve quality of life without curing the disease.
Risk factors
Because of how rare CCK is, there has been limited research done into the riskthe possibility that something bad will happen factors of this disease.
Symptoms
CSSK may appear asymptomatic in the early stages of disease. As the tumour progresses, some of the following symptoms may appear:
- Abdominal massa growth of cells that come together to make a lump, may or may not be cancer, which may or may not be painful.
- Haematuriathe presence of blood in urine.
- Fever.
- Vomiting.
- Constipationa condition where a person has difficulty passing faeces/stools.
- Hypertensionhigh blood pressure.
- Anaemiaa condition where there aren't enough red blood cells in the blood, causing fatigue, weakness and pale skin and affecting how the body responds to infection.
- Fatiguea state of extreme tiredness or exhaustion, can be physical or mental.
Not everyone with the symptoms above will have cancer, but see your general practitioner (GP) if you are concerned.
Diagnosis
If your doctor suspects you have CSSK, they may order the following tests to confirm the diagnosisthe process of identifying a disease based on signs and symptoms, patient history and medical test results and refer you to a specialist for treatment:
- Physical examinationan examination of your current symptoms, affected area(s) and overall medical history.
- Imagingtests that create detailed images of areas inside the body tests, potentially including:
- MRI (magnetic resonance imaging)a type of medical imaging that uses radiowaves, a strong magnet and computer technology to create detailed images of the body.
- CT (computed tomography) scana type of medical imaging that uses x-rays and computer technology to create detailed images of the body.
- Ultrasounda type of medical imaging that uses soundwaves to create detailed images of the body .
- Blood teststesting done to measure the levels of certain substances in the blood.
- Biopsyremoval of a section of tissue to analyse for cancer cells.