What are the other Names for this Condition? (Also known as/Synonyms)

• CCS of Soft Tissue
• Clear Cell Sarcoma of the Tendons and Aponeuroses
• Malignant Melanoma of the Soft Parts

What is Clear Cell Sarcoma of Soft Tissue? (Definition/Background Information)

• Clear Cell Sarcoma (CCS) of Soft Tissue is an infrequent, aggressive, but often rapidly developing tumor, without signs and symptoms in the early stages. The tumor occurs in the soft tissues, especially in the tendons and aponeurosis (flat, fibrous membranes that anchor muscles)
• They are regarded as a kind of high-grade malignant cancer that spreads and infiltrates the surrounding tissues, tendons, muscles, and organs. The outcome for an individual with this tumor is poor, due to its high metastasizing rate
• CCS of Soft Tissue is seen to peak during mid-adult phase (ages 20-40 years). These tumors are normally located deep inside the body tissues, with common sites being the upper and lower limbs. Sometimes, the tumor extends into the subcutaneous region too
• A combination of chemotherapy, radiation therapy, and surgical procedures, are used to treat Clear Cell Sarcoma of Soft Tissue

Who gets Clear Cell Sarcoma of Soft Tissue? (Age and Sex Distribution)

• Clear Cell Sarcoma of Soft Tissue affects a wide range of age group, beginning from age 10 years. But, it normally peaks around age 30; most cases occurring, before the age of 40 years
• Infants, young children, and elderly adults, are usually not affected
• Females are affected slightly more than males. However, due to the rarity of CCS, this statistic keeps getting altered, as newer cases come up
• There is no known ethnic or racial preference

What are the Risk Factors for Clear Cell Sarcoma of Soft Tissue? (Predisposing Factors)

The risk of Clear Cell Sarcoma of Soft Tissue is linked to certain genetic mutations caused by reciprocal chromosomal translocations, where an exchange of genetic material takes place between two chromosomes.

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Clear Cell Sarcoma of Soft Tissue? (Etiology)

• The exact cause and mechanism of formation of Clear Cell Sarcoma of Soft Tissue, is unknown
• They are thought to occur due to genetic defects, occurring on account of certain chromosomal exchanges

What are the Signs and Symptoms of Clear Cell Sarcoma of Soft Tissue?

The signs and symptoms of Clear Cell Sarcoma of Soft Tissue include:

• The presentations are based on the location of CCS - in almost 90% of the cases, the upper and lower limbs (particularly the ankle joint, feet, and knee joint) are involved
• Other rarely affected regions include the head and neck and torso. Occasionally, the abdominal cavity, penis, spinal cord, nerve roots, are also affected
• The soft tissue tumors grow at a moderate rate and then suddenly start progressing rapidly. In most cases the initial periods are asymptomatic. CCS appears as a lump or mass that is initially painless; though, as the size increases, the tumors may be painful
• Due to large size of the tumor growth, the adjoining organs, nerves, and muscles, may be compressed or restricted. Often, these signs may be the first indications of the tumor
• In many individuals loss of weight and appetite, and fatigue, are observed. These symptoms appear later, as the cancer progresses

How is Clear Cell Sarcoma of Soft Tissue Diagnosed?

The diagnostic tests vary, based on location of the tumor. A diagnosis of Clear Cell Sarcoma of Soft Tissue may involve:

• Physical examination, evaluation of patient’s medical history
• Histopathological studies conducted on a biopsy specimen - the specimen is examined under a microscope by a pathologist, to arrive at a definitive diagnosis
• MRI scan, CT scan of the affected region, to aid in obtaining a clear image of the tumor, prior to surgery
• If tumor has metastasized (into the lungs or other regions) then PET scan, bone scan, and chest x-rays may be taken

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Clear Cell Sarcoma of Soft Tissue?

The complications of Clear Cell Sarcoma of Soft Tissue may include:

• Usually by the time CCS of Soft Tissue is detected, chances are that they would have proliferated and metastasized aggressively, damaging organs and tissues beyond repair. Frequent sites of metastasis are the lungs, lymph nodes, and bones
• Blood loss during invasive treatment methods may be heavy
• Damage of vital nerves, blood vessels, and surrounding structures, during surgery
• Side effects from chemotherapy (toxicity), radiation therapy

How is Clear Cell Sarcoma of Soft Tissue Treated?

The treatment of Clear Cell Sarcoma of Soft Tissue is undertaken as:

• Any combination of chemotherapy, radiation therapy, and invasive procedures are used to treat Clear Cell Sarcoma of Soft Tissue
• Embolization is used to provide temporary relief from the symptoms, and reduce blood loss during a surgical procedure
• Wide surgical excision of CCS, removal of the entire lesion, followed by radiation therapy (or chemotherapy) is the standard treatment mode. Local recurrences are generally unusual
• When CCS is at an inaccessible location, or is unsafe for surgical intervention, non-invasive procedures, such as chemotherapy and radiation therapy are adopted
• Post-operative care is important: A minimum activity level is to be ensured, until the surgical wound heals
• Follow-up care with regular screening and check-ups are important
• It is hoped that certain targeted medical therapies can be developed, based on research regarding the genetic causes of CCS of Soft Tissue

How can Clear Cell Sarcoma of Soft Tissue be Prevented?

• Current medical research have not established a way of preventing Clear Cell Sarcoma of Soft Tissue
• Regular medical screening at periodic intervals with blood tests, scans, and physical examinations, are mandatory for those who have already endured the tumor, due to its high metastasizing potential and possibility of recurrence. Often several years of active vigilance is necessary

What is the Prognosis of Clear Cell Sarcoma of Soft Tissue? (Outcomes/Resolutions)

• Clear Cell Sarcoma of Soft Tissue is rare, but a high-malignancy cancer that is not completely understood. The long-term prognosis depends on a combination of factors, such as:
  • Age of the individual
  • Tumor stage at detection
  • Tumor size and location
  • Its ki-67 value - a protein found in cells that is a good indicator of cell activity. The ki-67 value is determined by a pathologist and is usually mentioned in the pathology report
  • Patient’s response to treatment and medical therapy
• Any tumor over 5 cm in size with tissue necrosis, projects the worst outcome. The long-term outcome is poor for most individuals (10-year survival rate is 33%), given the high metastasizing rate (50%) of the tumor

Additional and Relevant Useful Information for Clear Cell Sarcoma of Soft Tissue:

Clear Cell Sarcoma of Soft Tissue has no similarity with clear cell carcinoma of kidney, a high-grade children’s tumor.