What is CNS High-Grade Neuroepithelial Tumour with BCOR Alteration?
CNS High-Grade Neuroepithelial Tumour with BCOR alteration is a very rare and aggressive brain or spinal cord cancer. It mainly affects children and young adults. “CNS” refers to the central nervous system, which includes the brain and spinal cord.
“High-grade” means that the tumour grows quickly and tends to spread. “BCOR” is a gene that, when altered or mutated, causes the cells to grow abnormally and become cancerous. This tumour has been newly recognised and has been added to recent medical classifications based on its unique genetic alteration.
Symptoms
Patients with this tumour may start having symptoms depending on where the tumour is located. Common signs include headaches, nausea, vomiting, vision problems, balance issues, seizures, or weakness in different parts of the body. In children, parents may also notice personality changes, school difficulties, or delayed development.
Diagnosis
To diagnose this condition, doctors use brain imaging tests like MRI or CT scans to locate the tumour. A sample of the tumour (biopsy) is examined under a microscope, and special genetic tests are performed to detect the BCOR alteration. This helps doctors confirm the exact type of tumour.
Treatment
Treatment usually begins with surgery to remove as much of the tumour as possible. However, complete removal is often hard because the tumour can be located deep within the brain or spinal cord. After surgery, chemotherapy and radiation are given to kill the remaining cancer cells. In some cases, doctors may suggest joining clinical trials for new treatments targeting BCOR mutations.
Prognosis
The prognosis for this tumour is generally poor, as it tends to grow fast and come back even after treatment. But research is ongoing, and doctors are working on targeted therapies that may improve survival. Regular monitoring and supportive care are important for managing this challenging condition.
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