What is CIC-Rearranged Sarcoma?
CIC-Rearranged Sarcoma is a very rare and aggressive type of soft tissue cancer. It develops because of a specific genetic change involving a gene called CIC. In this condition, the CIC gene becomes abnormally joined with another gene, creating what is called a “gene rearrangement.” This abnormal fusion causes cells to grow and divide uncontrollably, leading to tumour formation.
For many years, this tumour was thought to be a type of Ewing sarcoma because it looks similar under the microscope. However, modern genetic testing has shown that it is a completely separate and more aggressive disease. It is now recognised as a distinct category of sarcoma.
Incidence
CIC-rearranged sarcoma is extremely rare. It most commonly affects young adults and adolescents, but it can occur at any age. It usually develops in the soft tissues, especially in the arms, legs, or trunk of the body. In some cases, it may arise in deeper tissues, which can delay detection.
Because it is so uncommon and resembles other tumours, accurate diagnosis requires specialised genetic testing.
Symptoms
The most common symptom is a growing lump in the soft tissue. At first, the lump may be painless, which can cause delay in seeking medical attention. As the tumour increases in size, it may cause pain, swelling, or tightness in the surrounding area. If located near joints or muscles, it can lead to difficulty in movement. In advanced stages, general symptoms such as fatigue or weight loss may appear.
This tumour tends to grow faster than many other soft tissue sarcomas, and it has a higher tendency to spread to the lungs or other organs.
Diagnosis
Diagnosis begins with imaging tests such as MRI or CT scans to evaluate the size and location of the mass. A biopsy is essential to confirm the disease. Under the microscope, the tumour may appear similar to other small round cell tumours. Therefore, molecular testing is required to identify the specific CIC gene rearrangement. Without genetic confirmation, the diagnosis may be missed.
Additional scans are performed to check whether the cancer has spread to other parts of the body.
Treatment
Treatment usually involves a combination of surgery, chemotherapy, and sometimes radiation therapy. Surgery aims to remove the tumour completely. Chemotherapy is often given because this tumour behaves aggressively and has a higher risk of spreading. Radiation may be added to reduce the risk of local recurrence.
Because it is more aggressive than similar sarcomas, treatment plans are often intensive.
Prognosis
The prognosis of CIC-rearranged sarcoma is generally more serious compared to Ewing sarcoma and some other soft tissue cancers. It has a higher chance of recurrence and metastasis, especially to the lungs. However, early diagnosis and complete treatment improve survival chances. Regular follow-up is extremely important to monitor for recurrence.
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