glioma



Overview of Neuroglial Tumor

Tumors originating from glial cells, most of which are malignant. Common symptoms include headache, nausea and vomiting, hypesthesia, and motor sensory deficits. Surgery is the mainstay of treatment, and combined with radiotherapy, chemotherapy, and targeted therapies, the overall prognosis is poor, but aggressive treatment can improve the prognosis.

What is Glioma?

Definition

Glioma is a collective term for tumors that originate from glial and neuronal cells of the nervous system.

Gliomas can occur in the brain and spinal cord, with the vast majority occurring in the brain, and are the most common intracranial malignant tumor.

Depending on the cell type, gliomas can be classified as astrocytomas, glioblastomas, oligodendrogliomas, etc. The treatment and prognosis of different types of gliomas also vary.

Typing

Classification according to histologic and molecular pathologic features

Adult-type diffuse glioma

Childhood-type diffuse low-grade glioma

Childhood-type diffuse high-grade glioma

Limited astrocytic glioma

Ventricular meningeal tumors

Classification by cell morphology

Astrocytoma

Oligodendroglioma

Ventricular Mural Tumor

Mixed Glioma

Classification by Tumor Location

Supratentorial glioma

Subtentorial Glioma

Brainstem Glioma

Spinal cord glioma

Incidence

There are 5 to 8 new cases of gliomas per 100,000 people in China each year.

The incidence of different types of gliomas varies.

Astrocytoma is 0.5/100,000～1/100,000

0.2/100,000 to 0.8/100,000 for ventricular meningiomas

Oligodendroglioma is about 0.27/100,000

Mixed glioma is 0.19/100,000

The age of onset is mostly between 21 and 50 years old, with a peak between 31 and 40 years old, and it is also more common in children around 10 years old.

There are more males than females.

The 5-year mortality rate is high, second only to pancreatic cancer and lung cancer among systemic tumors.

Questions you may be concerned about

Are gliomas benign or malignant?

Most gliomas are malignant tumors.

Clinically, gliomas are classified into astrocytomas, oligodendrogliomas, ventricular meningiomas, and mixed gliomas based on tumor morphology, and each

According to the degree of malignancy of the tumor, gliomas can be classified as grade I, II, III and IV. Among them, grade I is benign tumors and the rest are low malignant and malignant tumors.

Among the many types of gliomas, only hairy cell type astrocytic and subventricular giant cell astrocytomas belong to WHO grade Ⅰ, and the rest are low-grade malignant and malignant tumors. Therefore, most gliomas are malignant.

How long can I live with a glioma?

Most gliomas are malignant and have a poor overall prognosis.

Patients with gliomas progress more rapidly and may recur within a short period of time after surgery, and the survival time tends to be relatively short.

According to the degree of malignancy of the tumor, it is classified as grade I, II, III and IV.

When a glioma is WHO grade Ⅰ to Ⅱ, it can generally survive for 8 to 10 years.

WHO grade III gliomas can generally survive for 3 to 4 years.

WHO grade IV gliomas generally survive 14.6 to 17 months.

How effective is gene therapy for glioma?

The effectiveness of gene therapy for glioma is not known.

Gene therapy is a method of treating tumors by implanting external genetic material into tumor cells to produce novel substances.

Currently, there are 2 main directions of gene therapy for glioma: to induce apoptosis of tumor cells by herpes simplex virus thymidine kinase, or to reduce the growth and angiogenesis of glioma by RNA interference technology.

The above studies are still in the process of animal experiments or pre-tests, and have not entered the clinic, so the effect on humans is not yet clear.

Causes

Causes

The cause of the disease has not been clearly defined, and the two risk factors that have been identified so far are exposure to high doses of ionizing radiation and high exonic rate genetic mutations associated with rare syndromes.

Risk factors

Age: The risk of developing gliomas increases with age.

Exposure to radiation

People exposed to ionizing radiation have an increased risk of developing glioma.

Ionizing radiation includes radiation therapy used to treat cancer and certain radiation exposures caused by radioactive environments.

There is no clear evidence that electromagnetic fields from power lines and radiofrequency radiation from microwave ovens increase the risk of glioma.

It is not clear whether cell phone use increases the risk of glioma.

Family history of glioma: Having a family history of glioma doubles the risk of developing glioma.

Nitrite foods.

Bacterial or viral infections, such as glioblastoma associated with cytomegalovirus infection.

Head trauma.

Symptoms

Main Symptoms

Symptoms of patients with glioma are related to the location and size of the tumor, and the performance varies, the main symptoms are as follows.

Headache

Mostly manifested as aggravated by waking up in the morning, coughing and bowel movement.

It may be relieved temporarily after vomiting.

Nausea or vomiting

Especially common in children.

Most often comes in spurts in the early morning.

Hyperalgesia

A decrease in sensory sensitivity and a decrease in the ability to feel external stimuli.

For example, strong pain, hot or cold stimuli may cause only mild sensation or even no sensation at all.

Mental retardation

Loss of ability to calculate, read, speak, etc.

Mental disorder

Such as personality change or irritability.

Motor Disorders

Such as weakness, loss of balance, or even paralysis.

Urinary incontinence

Loss of self-control of urination and involuntary flow of urine.

Visual impairment

Loss of vision.

Loss of visual field and blurred vision.

Blindness may occur in severe cases.

Seizures.

The occurrence of epilepsy and the type of seizures are related to the site of the tumor.

They are mainly characterized by rhythmic muscle convulsions throughout the body, and in severe cases, loss of consciousness may occur.

Complications

Cerebral edema

Most common, it can be caused either by the tumor itself or by surgery or radiation therapy.

Early symptoms are nausea, vomiting, drowsiness or unresponsiveness. In severe cases, irregular or sudden respiration and coma may occur.

Consultation

Department of Medicine

Neurosurgery

Prompt medical attention is recommended if symptoms such as unrelieved or recurring headache, nausea or vomiting, physical weakness, blurred vision, etc. occur.

Neurology

You may also visit the Department of Neurology if you experience any of the above symptoms.

Emergency Department

If you experience severe symptoms such as sudden onset of convulsions, loss of consciousness, or paralysis, we recommend that you consult the Emergency Department as soon as possible or call the 120 emergency number.

Preparation

Information on how to register, preparation of documents, and frequently asked questions.

Tips for seeking medical treatment

If the patient is convulsing all over the body, remove dangerous objects from the surrounding area and do not forcefully pry open the mouth or stuff towels or chopsticks in the patient’s mouth.

Avoid applying painkillers on your own before going to the doctor, so as not to aggravate the symptoms or cover up the condition.

Special Note: When the headache is severe, it is recommended that family members accompany the patient to seek medical treatment, and avoid driving or riding on your own to seek medical treatment.

Preparation Checklist

What is the nature of the headache? How long has it lasted?

Has nausea or vomiting occurred?

Is there any limb movement disorder?

Have there been any generalized convulsions?

Can you see clearly? Is vision intact?

Has anyone in the family had a glioma?

Any recent bacterial or viral infections?

Has there been any head trauma?

Cranial CT, cranial MRI, PET-CT

Analgesics: aspirin, acetaminophen, ibuprofen

Others: valproate, oxcarbazepine, bevacizumab

Diagnosis

Diagnosis is based on

medical history

Family history of glioma.

History of bacterial or viral infections.

History of head trauma.

Clinical manifestations

Symptoms such as headache, nausea, vomiting, hyperalgesia, mental retardation, personality changes, irritability, generalized weakness, urinary incontinence, vision loss, and seizures.

Imaging

CT

CT mainly shows the density difference between glioma lesions and normal brain tissue, characteristic density manifestations such as calcification, hemorrhage and cystic changes, etc., the site of lesion involvement, edema and occupying effect.

CT is the most widely used diagnostic technique at present, which is convenient and fast, but the imaging of brain and spinal cord is not as clear as MRI.

MRI

MRI can mainly show the difference in signal intensity of hemorrhage, necrosis and edema of glioma and its occupying effect, and it can also show the invasive range of the lesion.

Multimodal MRI can not only reflect the morphological characteristics of glioma, but also the functional and metabolic status of the tumor tissue.

MRI examination takes a long time and requires patients’ cooperation, and some patients may experience discomfort.

Critically ill patients, patients wearing pacemakers or with ferromagnetic substances in the body cannot undergo this examination.

PET-CT

When a glioma is suspected and a biopsy is proposed, PET-CT can be used to identify the area of the lesion with the highest metabolic activity.

It can be helpful in identifying tumor recurrence from radionecrosis.

PET-CT can also rule out the possibility of metastasis to the brain from a tumor elsewhere in the body.

Pathologic examination

Pathological diagnosis of gliomas through tumor resection or biopsy is the “gold standard” for diagnosis of gliomas.

Currently, the main molecular pathologic markers include isocitrate dehydrogenase mutations, telomerase reverse transcriptase promoter mutations, and combined chromosome 1p/19q deletion status.

Biopsies can be categorized into stereotactic or navigated biopsies and open surgical biopsies.

Stereotactic or subnavigational biopsy is indicated for lesions with a much deeper location; whereas craniotomy biopsy is indicated for lesions with a superficial location or close to the cortex of the functional area.

Stereotactic puncture biopsy is usually guided by a CT or MRI scan, and pathology is performed after obtaining a small amount of brain tissue.

Tumor Classification

The World Health Organization Central Nervous System Tumor Classification divides gliomas into grades Ⅰ to Ⅳ, with grades Ⅰ and Ⅱ being low-grade gliomas and grades Ⅲ and Ⅳ being high-grade gliomas.

Grade I: Under a microscope, tumor cells look more like normal cells and grow and spread more slowly than grade II, III and IV tumor cells. They rarely spread into nearby tissues. A cure can be achieved if they are completely removed by surgery.

Grade II: Tumor cells grow and spread more slowly than grade III and IV tumor cells. They may spread into nearby tissues and may recur or progress to higher grade tumor forms.

Grade III: Under a microscope, tumor cells look very different from normal cells and grow faster than grade I and II tumor cells. They are likely to spread into nearby tissues.

Grade IV: Under a microscope, tumor cells do not look like normal cells and can grow and spread rapidly.

Treatment

Principles of Treatment

Glioma treatment is based on surgical resection, combined with a combination of radiotherapy and chemotherapy.

The determination of the treatment plan requires the participation of multiple disciplines, the adoption of individualized comprehensive treatment, the optimization and standardization of the treatment plan, with a view to achieving the maximum therapeutic benefits.

Surgical treatment

The principle of surgical treatment for glioma is maximum safe resection, and the surgical treatment can be mainly divided into tumor resection and pathological biopsy.

Tumor resection

Indications

CT or MRI suggests intracranial occupation.

There are obvious signs of intracranial hypertension and cerebral hernia.

Presence of neurological dysfunction due to tumor localization.

History of definite epileptic seizures.

Contraindication

Patients with severe cardiac, pulmonary, hepatic, renal dysfunction and recurrence, and poor general condition who cannot tolerate surgery.

Other contraindications that make it unsuitable to undergo neurosurgical craniotomy.

Biopsy

Indications

Elderly patients or suffering from serious co-morbidities.

Poor preoperative neurological status.

Tumor located in the dominant hemisphere, extensive infiltrative growth or invasion of both hemispheres.

The tumor is located in the cortex of the functional area, deep white matter or brainstem area, and cannot be satisfactorily resected.

The nature of the lesion needs to be identified.

Contraindications

Severe cardiac, pulmonary, hepatic, renal dysfunction and recurrent patients with poor general condition who cannot tolerate surgery.

Other contraindications that make the patient unsuitable for neurosurgery.

Application of new surgical assistive technology

The application of new surgical assistive technology can help determine the degree of surgical resection and tumor boundary and protect the function during surgery.

Neuroimaging navigation, functional neuroimaging navigation, intraoperative neurophysiological monitoring technology, intraoperative MRI real-time imaging neuronavigation, fluorescence-guided microsurgery, intraoperative ultrasound imaging real-time localization.

Multimodal neuronavigation combined with intraoperative cortical and subcortical localization can further improve surgical safety.

Determination of the degree of surgical resection of glioma

MRI should be reviewed within 24 to 72 hours after surgery for glioma and used as a basis for determining the efficacy of subsequent treatment or tumor progression.

Chemotherapy

According to the results of histopathology and molecular pathology, choose the appropriate chemotherapy regimen.

Chemotherapy should be carried out on the basis of maximum safe resection of the tumor.

Chemotherapy should be initiated and administered in adequate doses as early as possible after surgery.

Commonly used chemotherapy regimens for high-grade gliomas are Stupp regimen of temozolomide (TMZ), PCV regimen of methylbenzylhydrazine (PCB) + lomustine (CCNU) + vincristine (VCR).

Other drugs used in glioma treatment include carmustine, irinotecan, etoposide, cisplatin, carboplatin, and cyclophosphamide.

Chemotherapy for low-grade gliomas is controversial and should be actively considered in patients with low-grade gliomas who have high-risk factors.

There is no standard chemotherapy regimen for recurrent gliomas.

Radiation therapy

Radiation therapy is usually given after the pathologic diagnosis has been made.

Three-dimensional conformal (3D-CRT) or conformal intensity-modulation techniques (IMRT) are recommended.

Stereotactic radiotherapy (SRT) is not indicated for the primary treatment of gliomas.

Radiotherapy is recommended to be started as early as possible after surgery (2 to 6 weeks after surgery) for high-grade gliomas.

Synchronized radiotherapy is recommended for glioblastoma multiforme (GBM) and mesenchymal glioma.

Radiotherapy should be started as early as possible for low-grade gliomas with high-risk factors (age ≥40 years, incomplete resection of the tumor, large tumor size, preoperative neurological deficits, and IDH wild-type, etc.).

Partially resected ventricular meningiomas and mesenchymal ventricular meningiomas can be treated with radiotherapy.

For recurrent gliomas the safety of re-radiotherapy for recurrent gliomas should be evaluated.

Symptomatic treatment

Cerebral edema, increased intracranial pressure

One of the most common symptoms in the management of gliomas.

Hyperosmolar dehydrating agents (mannitol, glycerofructose, and albumin, among others), diuretics, and adrenocorticotropic hormones are currently the most commonly used treatments for cerebral edema, and can be used under medical supervision.

Glioma with seizures

Antiepileptic drugs may be administered under medical supervision.

Sodium valproate is most commonly used intravenously, and sodium valproate, oxcarbazepine and levetiracetam are available orally.

Acute Radiation Brain Injury

The main signs are intracranial hypertension, such as nausea, vomiting, headache and drowsiness, etc. Symptoms are usually transient and reversible.

Symptoms are usually short-lived and reversible. Adrenocorticotropic hormones can be used under medical supervision to relieve symptoms.

There is usually no effective treatment for advanced radiation brain damage.

Patients with gliomas in the terminal phase (last week to 3 months of life)

Often the intracranial pressure rises progressively and the patient suffers headaches, which may be accompanied by generalized pain. In terminal patients who are still conscious and emotional, pain relief is the primary therapeutic goal, and analgesic treatments are taken in a stepwise manner.

Electric field therapy

A treatment method that exerts anti-tumor effects by inhibiting mitosis of tumor cells.

The Electric Field Therapy System for gliomas is a portable device that generates a mid-frequency, low-field intensity tumor therapy magnetic field through a transducer sheet that is applied to the scalp.

It is recommended for the treatment of new glioblastoma multiforme and recurrent high-grade gliomas.

Targeted Therapy

Bevacizumab is a targeted drug used for recurrent glioblastoma multiforme that achieves anti-tumor effects by blocking tumor angiogenesis.

The effect on other gliomas is not clear.

Prognosis

Cure

Gliomas generally have a poor prognosis, with most high-grade gliomas recurring after initial treatment.

Approximately 70% of low-grade tumors (WHO grade II) will progress to high-grade tumors within 5 to 10 years.

The 1-year and 5-year survival rates for adult high-grade gliomas are approximately 30% and 13%, respectively.

The median survival times for mesenchymal gliomas and glioblastoma multiforme are approximately 2 to 3 years and 1 year, respectively.

Diffuse pontine gliomas occur predominantly in children, usually with onset between the ages of 5 and 7 years, and the median survival time for children is less than 12 months.

Survival of cancer patients can be broadly assessed using the 5-year survival rate.The 5-year survival rate is the percentage of patients who survive for more than 5 years after various combinations of treatments.

The probability of recurrence of the tumor after 5 years of radical treatment is very low, and it can generally be regarded as a clinical cure.

Special reminder: The 5-year survival rate is derived from a large sample of epidemiologic surveys and clinical studies, and does not represent the specific survival period of an individual. Individual patient survival is influenced by a variety of factors and cannot be predicted.

Prognostic Factors

The prognosis of patients with gliomas is related to the grade and molecular subtype of the tumor.

Prognostic factors associated with high-grade gliomas after surgery

Age (younger patients ≤65 years of age have a better prognosis than older patients >65 years of age).

Degree of tumor resection (total resection has a better prognosis than incomplete resection).

Site of the lesion (prognosis is better for focal gliomas than for multiple diffuse gliomas, and for lobar gliomas than for deep gliomas).

Primary or recurrent.

Poor prognostic factors for low-grade gliomas

Histology is diffuse astrocytoma.

Age ≥40 years.

Maximum tumor diameter ≥ 6 cm.

Tumor growth across the midline.

Presence of more than mild preoperative neurologic deficits.

Only 1 or no deletion of chromosome 1p/19q.

Daily

Daily Management

Dietary management

Maintain balanced nutrition, eat more lean meat, eat more fruits, vegetables and cereals with high fiber content, supplement enough water and keep the bowel movement smooth.

During radiotherapy, appetite may be affected, and even nausea, vomiting and other digestive adverse reactions may occur. Avoid eating greasy, spicy and overly sweet food, and choose a light diet that is easy to digest as much as possible.

Quit smoking and drinking, and do not drink strong tea and coffee.

Psychological support

Maintain an optimistic state of mind, communicate more with patients and family members to enhance confidence, and seek help from a psychiatrist if necessary.

Relax your body and mind through music, hypnosis, meditation and yoga.

Life management

Take sufficient rest and do not stay up late.

Avoid possible risk factors leading to glioma, such as too much and too close use of cell phone, reduce exposure to environment beyond normal radiation.

Exercise appropriately to enhance physical fitness and take care to prevent infection.

Continue to take relevant medications as prescribed by your doctor.

Functional Rehabilitation

Physical therapy: exercise therapy, including correct body position, joint mobility exercises, muscle strength training, endurance training, neuromuscular facilitation techniques, balance and coordination training, gait training and respiratory training.

Occupational therapy: basic occupational therapy necessary for daily living, value-creation occupational therapy, recreational or leisure occupational therapy, educational occupational therapy, and training in the use of assistive devices.

Speech therapy: Speech training, listening comprehension training, oral expression training, etc., as well as training in the use of sign language, drawings, communication boards, communication manuals, and computerized communication devices.

Swallowing Disorder Treatment: It mainly includes the change of nutritional intake pathway, rehabilitation training to promote the recovery of swallowing function, adjustment of food properties and eating position, and rehabilitation care and education related to swallowing rehabilitation.

Cognitive and behavioral therapy: including education to enhance the awareness and understanding of cognitive deficits, adaptive therapy to reduce the impact of cognitive deficits, and restorative therapy for cognitive deficits.

Rehabilitation engineering: ankle-foot orthoses to improve foot drop, wide-base quadrupedal canes, standard walkers or semi-walkers to increase the support surface to reduce the risk of falling when walking or standing.

Follow-up and review

Follow up and review regularly as prescribed by the doctor.

Follow-up: Patients should undergo a review of their basic clinical conditions, including general condition, cognitive and psychosomatic status, neurological signs and physical examination, necessary auxiliary examinations, and imaging review.

The routine follow-up interval for patients with high-grade glioma is 1 to 3 months; for low-grade glioma, it is 3 to 6 months.

Adults with low-grade gliomas should be followed up every 3 to 6 months for 5 years; thereafter, they should be followed up at least once a year.

High-grade gliomas should be followed up once every 2 to 6 weeks after radiotherapy, and then every 1 to 3 months for 2 to 3 years, and then the follow-up interval can be extended appropriately.

Adult intracranial ventricular meningiomas should be followed up every 3 to 4 months for 1 year; then every 4 to 6 months for 2 years; and then every 6 to 12 months thereafter.

Prevention

The etiology of glioma is still unclear, and there is no effective method of prevention. However, risk factors that cause glioma can be avoided to reduce the risk of developing the disease.

Maintain a healthy lifestyle and a normal weight.

Avoid unnecessary radiologic tests and stay away from radiation.

Those with a family history of glioma should have regular medical checkups.

Do not abuse hormonal drugs.

Pay attention to life safety and avoid trauma.

Pay attention to hygiene and wash hands frequently. Wear masks when going to crowded places to avoid viral infection.

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