Glioma
Disease Details
Family Health Simplified
- Description
- Glioma is a type of tumor that occurs in the glial cells of the brain or spine, which support and protect the nervous system.
- Type
- Glioma is a type of tumor that occurs in the glial cells of the brain or spine. Its genetic transmission is generally not inherited but rather results from somatic mutations that occur during a person's lifetime. In rare cases, some genetic syndromes, like Li-Fraumeni syndrome or Neurofibromatosis type 1, can increase the risk of developing gliomas, implying a hereditary component for those specific cases.
- Signs And Symptoms
- Symptoms of gliomas depend on which part of the central nervous system is affected. A brain glioma can cause headaches, vomiting, seizures, and cranial nerve disorders as a result of increased intracranial pressure. A glioma of the optic nerve can cause vision loss. Spinal cord gliomas can cause pain, weakness, or numbness in the extremities. Gliomas do not usually metastasize by the bloodstream, but they can spread via the cerebrospinal fluid and cause "drop metastases" to the spinal cord. Complex visual hallucinations have been described as a symptom of low-grade glioma.A child who has a subacute disorder of the central nervous system that produces cranial nerve abnormalities (especially of cranial nerve VII and the lower bulbar nerves), long-tract signs, unsteady gait secondary to spasticity, and some behavioral changes is most likely to have a pontine glioma.
- Prognosis
- Prognosis of gliomas is given in relation to what grade (as scored by the World Health Organization system) of tumour the patient presents with. Typically, any tumour presenting as above WHO grade I (i.e. a malignant tumour as opposed to a benign tumour) will have a prognosis resulting in eventual death, varying from years (WHO grade II/III) to months (WHO grade IV). Prognosis can also be given based on cellular subtype, which may also impact prognosis.
- Onset
- Glioma is a type of tumor that occurs in the brain and spinal cord. The onset of gliomas can vary significantly; they may develop and produce noticeable symptoms over a few months, but some may only be detected incidentally or after significant growth. Symptoms can include headaches, seizures, cognitive or personality changes, and neurological deficits such as weakness or speech difficulties. These symptoms often prompt medical evaluation and imaging studies, which can lead to the diagnosis of glioma.
- Prevalence
- Gliomas are relatively common primary brain tumors, accounting for about 30% of all brain and central nervous system tumors and 80% of all malignant brain tumors. The incidence rate is approximately 6 cases per 100,000 people annually. Prevalence can vary based on the specific type of glioma and demographic factors.
- Epidemiology
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Gliomas are a type of tumor that occurs in the brain and spinal cord, originating from glial cells. They are among the most common primary brain tumors. Here is a summary of the epidemiology of gliomas:
- **Incidence**: Gliomas account for roughly 30% of all brain and central nervous system (CNS) tumors and 80% of all malignant brain tumors.
- **Age**: Gliomas can occur at any age but are most commonly diagnosed in adults between the ages of 45 and 65. Pediatric gliomas, including those in infants, also occur and are classified differently.
- **Gender**: Gliomas are generally more common in males than females.
- **Geography**: The incidence rates of gliomas vary globally, with slightly higher rates observed in Europe and North America compared to Africa and Asia.
- **Risk Factors**: Known risk factors include genetic conditions (such as neurofibromatosis types 1 and 2, Li-Fraumeni syndrome, and Turcot syndrome), exposure to ionizing radiation, and a family history of gliomas. Most cases, however, have no clearly identifiable risk factors.
For more comprehensive details, consulting specific epidemiological studies or reviews can provide additional insights into the demographics, geographic distribution, and potential etiological factors associated with gliomas. - Intractability
- Gliomas are often challenging to treat due to their location in the brain and their potential to infiltrate surrounding tissues. While they are not universally intractable, the prognosis and treatment outcomes can vary widely depending on the type, grade, and specific characteristics of the glioma. Higher-grade gliomas, such as glioblastomas, are generally more difficult to treat successfully and have a poorer prognosis compared to lower-grade gliomas. Treatment options typically include a combination of surgery, radiation therapy, and chemotherapy, but the effectiveness can be limited, and recurrence is common.
- Disease Severity
- Glioma severity can vary widely depending on the type and grade of the tumor. Low-grade gliomas (grades I and II) tend to grow more slowly and may have a better prognosis, while high-grade gliomas (grades III and IV) are more aggressive and have a poorer prognosis. Factors influencing severity include tumor size, location, and the patient's overall health.
- Healthcare Professionals
- Disease Ontology ID - DOID:3070
- Pathophysiology
- High-grade gliomas are highly vascular tumors and have a tendency to infiltrate diffusely. They have extensive areas of necrosis and hypoxia. Often, tumor growth causes a breakdown of the blood–brain barrier in the vicinity of the tumor. As a rule, high-grade gliomas almost always grow back even after complete surgical excision, so are commonly called recurrent cancer of the brain.Conversely, low-grade gliomas grow slowly, often over many years, and can be followed without treatment unless they grow and cause symptoms.Several acquired (not inherited) genetic mutations have been found in gliomas. Tumor suppressor protein 53 (p53) is mutated early in the disease. p53 is the "guardian of the genome", which, during DNA and cell duplication, makes sure the DNA is copied correctly and destroys the cell (apoptosis) if the DNA is mutated and cannot be fixed. When p53 itself is mutated, other mutations can survive. Phosphatase and tensin homolog (PTEN), another tumor suppressor gene, is itself lost or mutated. Epidermal growth factor receptor, a growth factor that normally stimulates cells to divide, is amplified and stimulates cells to divide too much. Together, these mutations lead to cells dividing uncontrollably, a hallmark of cancer. In 2009, mutations in IDH1 and IDH2 were found to be part of the mechanism and associated with a less favorable prognosis.
- Carrier Status
- Carrier status is not applicable to glioma. Gliomas are a type of tumor that occurs in the brain and spinal cord and are not inherited in a way that has a carrier status, unlike some genetic diseases. Instead, gliomas develop due to a combination of genetic mutations and environmental factors.
- Mechanism
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Glioma is a type of tumor that occurs in the glial cells of the brain or spine. Here's an overview of its basic and molecular mechanisms:
**Mechanism:**
Gliomas can arise from various types of glial cells, including astrocytes, oligodendrocytes, and ependymal cells. These tumors are classified based on their cell type and grade, which ranges from low grade (I and II) to high grade (III and IV, with grade IV glioblastoma being the most aggressive form).
**Molecular Mechanisms:**
1. **Genetic Mutations:**
- **IDH1 and IDH2 mutations:** Common in lower-grade gliomas and secondary glioblastomas. These mutations result in the production of an oncometabolite, 2-hydroxyglutarate, which can interfere with cellular differentiation and promote tumorigenesis.
- **TP53 mutations:** Often seen in astrocytomas and secondary glioblastomas, leading to loss of tumor suppressor function.
- **ATRX mutations:** Associated with alternative lengthening of telomeres, common in astrocytomas and oligodendrogliomas.
- **1p/19q co-deletion:** A hallmark of oligodendrogliomas, associated with better prognosis and response to therapy.
2. **Signal Pathway Alterations:**
- **EGFR amplification/mutation:** Particularly in primary glioblastomas, leading to enhanced cell proliferation and survival.
- **PTEN loss/mutation:** Often observed in glioblastomas, causing activation of the PI3K/AKT pathway, promoting cell survival and growth.
- **RTK/RAS/PI3K pathway alterations:** Involves mutations/amplifications in receptor tyrosine kinases (like PDGFRA), RAS signaling proteins, and PI3K components.
3. **Epigenetic Modifications:**
- **MGMT promoter methylation:** Methylation of the MGMT promoter is associated with better response to alkylating agents like temozolomide in glioblastoma patients.
- **Histone modifications:** Can lead to changes in gene expression that contribute to tumor progression.
4. **Tumor Microenvironment:**
- The glioma microenvironment, which includes immune cells, vasculature, and extracellular matrix, plays a crucial role in tumor development and progression.
- **Hypoxia:** Common in high-grade gliomas, leading to HIF-1α activation, promoting angiogenesis and survival under low oxygen conditions.
Understanding these mechanisms is crucial for developing targeted therapies and improving treatment outcomes for glioma patients. - Treatment
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Treatment for brain gliomas depends on the location, the cell type, and the grade of malignancy. Current treatment options include surgical removal, radiation (radiation therapy), and chemotherapy. In some cases, tumour treating fields (alternating electric field therapy), a recently developed technology, may be used.
Often, treatment is a combined approach, using surgery, radiation therapy, and chemotherapy. For many, treatment consists of just surgery, or even "watchful waiting" (waiting to see when an intervention is justified due to tumour progression). Doctors carefully balance the specifics of the patient's tumour and the downsides of intervention, since there can be significant side effects from medical intervention.
Radiation and chemotherapy remain the mainstays of treatment beyond surgery. Radiation therapy is delivered in the form of external beam radiation or the stereotactic approach using radiosurgery. Temozolomide is a common chemotherapy drug which can be administered easily in an outpatient setting and is able to cross the blood–brain barrier effectively.
There are a wide variety of novel treatments currently being tested in clinical trials, ranging from IDH inhibitors like Ivosidenib, to the recently approved Dendritic cell-based cancer vaccine approach. Treatment using immunotherapy is another promising research path that may help treat glioma in the near future. Experimental therapies like oncolytic viruses have shown potential therapeutic benefits in clinical trials (but have not been approved for use in non-experimental settings). - Compassionate Use Treatment
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Compassionate use treatment for glioma refers to the use of investigational drugs outside of clinical trials for patients with serious or life-threatening conditions who have no other treatment options. These treatments often require special approval from regulatory agencies.
Off-label treatments for glioma include medications that are approved for other conditions but used to treat glioma based on emerging evidence or clinical experience. Examples include:
1. **Bevacizumab (Avastin)**: Originally approved for colorectal cancer, it is sometimes used off-label for recurrent glioblastoma.
2. **Temozolomide (Temodar)**: Though approved for glioblastoma, it is occasionally used off-label for other types of high-grade gliomas.
Experimental treatments are typically available through clinical trials and may include:
1. **Immunotherapies**: Such as vaccines (e.g., DCVax) and checkpoint inhibitors (e.g., nivolumab).
2. **Targeted therapies**: Like tyrosine kinase inhibitors (e.g., erlotinib).
3. **Gene therapies**: Involving the introduction of new genes into cells to fight cancer.
4. **Oncolytic virus therapies**: These use modified viruses to selectively infect and kill cancer cells.
Patients interested in off-label or experimental treatments should consult with their healthcare provider to discuss potential benefits and risks. - Lifestyle Recommendations
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For managing glioma, lifestyle recommendations include:
1. **Healthy Diet**: Eat a balanced diet rich in fruits, vegetables, lean proteins, and whole grains. Avoid highly processed foods and excessive sugar or fat intake.
2. **Regular Exercise**: Engage in regular physical activity, tailored to your ability and doctor's advice, to maintain overall health and reduce fatigue.
3. **Adequate Rest**: Ensure you get sufficient sleep and rest to help your body recover from treatments and manage stress.
4. **Stress Management**: Practice stress-reducing techniques such as meditation, deep breathing exercises, or yoga.
5. **Avoid Alcohol and Tobacco**: Refrain from smoking and limit alcohol consumption, as these can worsen health outcomes.
6. **Medication Adherence**: Follow your medical treatment plan precisely, including medications, therapies, and follow-up appointments.
7. **Support System**: Build a strong support system with family, friends, and support groups to help cope with emotional and psychological challenges.
Consult your healthcare provider before making any significant lifestyle changes to ensure they are safe and appropriate for your specific condition. - Medication
- Glioma treatment often includes medications such as corticosteroids to reduce inflammation and swelling, and anticonvulsants to manage seizures. Chemotherapeutic agents like temozolomide are commonly used. Bevacizumab, a monoclonal antibody, may also be prescribed to inhibit the formation of new blood vessels that supply the tumor.
- Repurposable Drugs
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For glioma, some repurposable drugs that have shown potential in various studies include:
1. **Metformin (commonly used for Type 2 Diabetes)**
2. **Chloroquine and hydroxychloroquine (antimalarial drugs)**
3. **Valproic acid (an antiepileptic drug)**
4. **Disulfiram (used for alcohol dependence)**
5. **Itraconazole (an antifungal drug)**
Information on "nan" was not clear in the context provided. If you meant nanotechnology, it involves the use of nanoparticles for targeted drug delivery, improving the efficacy and reducing the side effects of treatments for glioma. Some nanoparticle formulations under investigation include:
1. **Lipid-based nanoparticles**
2. **Polymeric nanoparticles**
3. **Gold nanoparticles**
4. **Iron oxide nanoparticles**
These technologies aim to deliver chemotherapeutic agents more effectively to the glioma cells. - Metabolites
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Glioma cells exhibit altered metabolism that supports their rapid growth and survival. Metabolites associated with glioma include:
1. **2-Hydroxyglutarate (2-HG)**: Especially in isocitrate dehydrogenase (IDH) mutant gliomas, 2-HG accumulates and acts as an oncometabolite.
2. **Lactate**: Elevated levels are seen due to increased glycolysis, known as the Warburg effect.
3. **Glutamine**: Glioma cells often increase glutamine consumption, which is used in various biosynthetic and bioenergetic pathways.
4. **Choline-containing compounds**: Increased levels are linked to cell membrane turnover and signaling.
Nanotechnology in glioma research focuses on:
1. **Nanoparticles**: These are used for targeted drug delivery, aiming to cross the blood-brain barrier more efficiently and deliver chemotherapeutic agents directly to tumor cells.
2. **Nano-imaging agents**: These enhance the visualization of gliomas during imaging techniques like MRI and CT scans, improving diagnosis and monitoring. - Nutraceuticals
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Currently, there is limited evidence on the use of nutraceuticals for glioma treatment. Some studies suggest that certain compounds like curcumin, resveratrol, and green tea polyphenols might offer supportive benefits, potentially enhancing the effectiveness of conventional therapies and providing antioxidant properties. However, these findings are preliminary, and more rigorous clinical trials are required.
Regarding nanotechnology, it holds promise in glioma treatment by enabling targeted drug delivery, improving drug accumulation in tumor cells, and reducing side effects. Nanoparticles can be engineered to cross the blood-brain barrier and deliver chemotherapeutic agents directly to glioma cells, thereby increasing treatment efficacy. Research is ongoing in this area to optimize these technologies for clinical use. - Peptides
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Glioma is a type of tumor that occurs in the glial cells of the brain or spine. Peptides can play a role in glioma treatment and diagnosis in several ways. They can be used in targeted therapy to deliver drugs specifically to tumor cells, minimizing damage to surrounding healthy tissue. Peptide vaccines are also being studied to stimulate the immune system to attack glioma cells.
Nanotechnology offers innovative approaches for glioma treatment and imaging. Nanoparticles can be engineered to cross the blood-brain barrier, delivering drugs directly to the tumor site. They can also be used for imaging, helping to better visualize tumors during diagnosis and treatment. Additionally, nanoscale materials can be used for photothermal therapy, where nanoparticles are used to convert light into heat to destroy tumor cells.