What Is SHH-Activated Medulloblastoma?
SHH-Activated Medulloblastoma is a type of medulloblastoma, a cancerous brain tumour that develops in the cerebellum, the part of the brain that controls balance and coordination. It occurs when the Sonic Hedgehog (SHH) signaling pathway, which normally helps brain development, becomes abnormal and causes cells to grow uncontrollably.
Causes
The exact cause is unknown. However, genetic changes affecting the SHH pathway can lead to the development of this tumour. Most cases occur randomly, although some may be associated with inherited genetic conditions.
Signs and Symptoms
Common symptoms include:
- Headaches, especially in the morning
- Nausea and vomiting
- Balance and coordination problems
- Difficulty walking
- Dizziness
- Blurred or double vision
- Fatigue and sleepiness
These symptoms usually result from increased pressure inside the skull caused by the growing tumour.
Diagnosis
Doctors may use the following tests to diagnose SHH-Activated Medulloblastoma:
- Neurological examination
- MRI scan of the brain and spine
- Examination of tumour tissue after surgery or biopsy
- Molecular testing to confirm the SHH subtype
- Cerebrospinal fluid (CSF) analysis to check for tumour spread
Treatment
Treatment usually includes:
Surgery
Removal of as much of the tumour as possible.
Radiation Therapy
High-energy radiation is used to destroy remaining cancer cells after surgery.
Chemotherapy
Anti-cancer drugs help kill cancer cells and reduce the risk of recurrence.
Targeted Therapy
Some patients may benefit from newer drugs that specifically target abnormalities in the SHH pathway.
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