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Medulloblastoma

Medulloblastoma is a brain tumor in children. It accounts for about 20% of all childhood brain tumors, being one of the most common tumor of the nervous system. Medulloblastomas are all classified as Grade IV tumors, they are fast-growing tumors that spread to other areas of the brain and spinal cord through cerebrospinal fluid (CSF). The cause of most medulloblastomas is not known. There’s a small percentage of childhood medulloblastomas that are related to gene changes that can run in families. There’s no known cause for adults diagnosed with medulloblastomas. Medulloblastoma survival rates depend on the patient’s age, clinical features of the tumor, and the spread of the tumor.

The HTT protein plays an important role in nerve cells (neurons) in the brain and is essential for normal post-embryonic development before birth. This mutation leads to an abnormally long version of the HTT protein that is cut into smaller, toxic fragments which bind together and accumulates in neurons, disrupting the normal functions of these cells and leading to neurodegeneration. HD commonly affects patients between the ages of 30 to 50 years. HD is not equally prevalent across the world and has a worldwide prevalence of only 2.7 per 100,000. It is most prevalent in the West as compared to Asian countries.

This variation is attributed to the difference in length of the HTT gene among different ethnicities. Males and females have almost the same risk of getting HD. If the onset of HD symptoms is in childhood, it is called juvenile HD. In the late-onset of HD, symptoms appear in the seventh or eighth decade of life.

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Symptoms

Symptoms related to a medulloblastoma depend on the tumor’s location.

  • Headaches

  • Nausea

  • Vomiting

  • Blurred and double vision

  • Extreme sleepiness

  • Confusion

  • Seizures and even passing out

  • Problems with walking, balance, and/or fine motor movements

  • Weakness or numbness in the arms and or legs

  • A change in normal bowel or bladder habits

  • Spinal pain

Diagnosis

The specialized imaging technique used to diagnose medulloblastoma are magnetic resonance imaging (MRI) and computed tomography (CT) scans., Also, histological examination of the tumor  through biopsy, are the major diagnostic tools.

Management

Medulloblastoma is commonly treated with surgical resection of the tumor followed by radiotherapy and chemotherapy. Physical therapy and physical training have evolved as an important therapeutic modality and have shown promising results in neuronal recovery of s patients with brain tumors, post-operatively.

Some major targeted therapeutics under clinical investigation are: The most extensively studied targeted therapeutics are SMO inhibitors, such as cyclopamine, vismodegib, and sonidegib. Particularly, sonidegib (NCT01708174) and vismodegib are currently in phase II clinical trials in patients showing relapse with SHH subgroup medulloblastoma.

Cancer vaccines clinical trial, currently in phases I and II, are based on a cancer vaccine consisting of RNA-loaded autologous dendritic cells which are administered to patients with MB (NCT01326104). This procedure is complicated and time-consuming as tumor cells from each patient have to be collected separately.

Many targeted therapeutics such as drugs and cancer vaccines for medulloblastomas are currently under trials and further research may be promising to pave way for better management and quality of life for patients with medulloblastoma.

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