Malignant meningioma



Overview

Definition

Meningioma is a type of growth from the meninges, most of which are benign.

Malignant meningioma is a type of meningioma in which malignant cells are present and may grow directly or progressively from benign or atypical meningiomas [1-2].

Staging or classification

Typing is mainly based on tumor cytopathology [1-2].

Mesenchymal meningioma: extremely malignant and the most common type of malignant meningioma.

Papillary type: prevalent in children and more aggressive.

Rhabdomyosiform type: less common.

Incidence

Meningiomas have an incidence of 4.7/100,000, accounting for approximately 30% of intracranial tumors and are the second most common central nervous system tumor [3-4].

Malignant meningiomas account for 1.0% to 3.0% of meningiomas, with peak incidence between 75 and 84 years of age, and commonly occur in men.

Etiology

Causes

The cause of malignant meningioma is still unclear, and may be related to genetic mutation, radiation, certain viruses and aflatoxin infection.

Pathogenesis

Malignant meningiomas are generally large in size, with obvious edematous reactions around the tumor. Tumors grow in different locations and ways, and the effects on brain tissue function and clinical manifestations vary widely [5-6]:

Direct compression, infiltration and destruction of the cranial brain functional area by the tumor can cause local neurological injury symptoms and epilepsy.

The occupying effect of the tumor itself or the obstruction of cerebrospinal fluid or venous sinus circulation by the tumor, resulting in obstructive hydrocephalus or obstruction of venous return, can cause symptoms of intracranial hypertension.

Symptoms

Malignant meningioma may have no obvious symptoms in the early stage and is often found after physical examination or trauma.

As the tumor grows, local nerve injury, epilepsy and cranial hypertension symptoms similar to benign meningioma gradually appear.

In addition, distant metastasis may also occur, manifesting as systemic symptoms.

Main symptoms

Localized neurological dysfunction

Tumors growing in different parts of the body can cause corresponding neurological dysfunction [7-8].

Tumor located in the anterior skull base: there may be decreased or loss of sense of smell, nasal obstruction, rhinorrhea, vision loss, visual field defects, headache, epilepsy and personality changes.

The tumor is located in the middle skull base: there may be loss of vision, visual field defects, protruding eyeballs, diplopia, facial numbness, restricted mouth opening, headache, epilepsy and psychiatric symptoms.

The tumor is located in the posterior skull base: it can cause endocrine function abnormalities, such as low or loss of sexual and reproductive function, polydipsia, bulimia, obesity and so on.

Tumor located in the convex side of the brain and sagittal sinus: it can cause neurological dysfunction such as limb paralysis and sensory disorder.

The tumor is located in the frontal lobe: it can present mental symptoms such as apathy, irritability, depression and delusion; it can present cognitive dysfunction such as executive ability, memory, decreased attention and dementia.

Epilepsy

When the tumor is located in the cerebral cortex, especially in the frontal and temporal lobes, it is easy to cause epileptic seizures, with various forms of manifestations.

It may manifest as generalized convulsions, cyanosis, foaming at the mouth, dilated pupils and loss of consciousness.

It can also manifest as localized convulsions, such as repetitive twitching of one side of the face, fingers or toes.

It can also manifest as involuntary activities, such as repeatedly fastening buttons, getting up, and opening doors.

Symptoms of cranial hypertension

Headache

Most often manifests as worsening on waking in the morning, coughing, straining and defecation.

It may be temporarily relieved by vomiting.

Vomiting

Nausea and vomiting: mostly in the early morning, more violent, jet-like episodes.

Systemic symptoms

Malignant meningioma may metastasize to the lung, pleura, breast, spine, liver and other parts of the body, manifesting symptoms related to the metastatic organs and systemic manifestations.

The performance of patients varies greatly among different metastatic foci, commonly such as cough, hemoptysis, breast swelling, skin ulceration and so on.

Generalized weakness, extreme emaciation, and non-infectious fever are advanced manifestations of the disease.

Complications

Cerebral edema/brain herniation

Large areas of cerebral edema may develop during tumor growth, and surgery or radiation therapy may also cause severe edema.

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

Infection

Upper respiratory tract and lung infections and urinary tract infections can be caused by prolonged bed rest, dysphagia, poor coughing and sputum ability, and untimely perineal care.

The manifestations are fever, cough, cough sputum, cloudy urine.

Pressure sores

Patients due to unfavorable limb movement, long-term bed-ridden caused by skin pressure damage.

The manifestations are redness, purple, blisters and ulceration of the skin at the pressure site.

Lower extremity deep vein thrombosis/pulmonary embolism

Lower extremity deep vein thrombosis may occur in patients who are paralyzed and bedridden for a long period of time.

It is characterized by swelling of the limb, slightly high local skin temperature, and in severe cases, distal necrosis of the limb.

Dislodgment of venous thrombus may cause pulmonary embolism, resulting in dyspnea, cyanosis, coughing, hemoptysis, etc., which is life-threatening.

Consultation

Department of Medicine

Neurosurgery

Imaging examination found intracranial occupation, or the appearance of epilepsy, headache, vomiting, limb movement disorder, aphasia and other symptoms should be promptly consulted.

Emergency Department

If you have sudden severe headache, vomiting, or unconsciousness, we recommend that you call 120 emergency or go to the Emergency Department.

Preparation

Consultation: Registration, Preparation of documents, Frequently Asked Questions

Tips for seeking medical treatment

Try to keep a record of the symptoms and duration of the onset of the illness so that you can give the doctor more information.

If you are going to be examined by other hospitals, be sure to bring along the relevant tests and medical records.

Patients with limited mobility, seizures or cognitive impairment are strongly advised to be accompanied by their family members, and not to drive or ride to the doctor on their own.

Preparation Checklist

Symptom checklist

Particular attention should be paid to the time of onset of symptoms, special manifestations, etc.

Is there headache, dizziness, seizure?

Is there any weakness, loss of sensation, numbness on one side of the limb?

Can you see clearly? Is vision intact?

Have similar symptoms occurred before?

List of medical history

Has there been any traumatic brain injury?

Has there been previous surgery for a meningioma? What was the pathology? Has there been any follow-up treatment?

Any neoplastic disease such as lung cancer, breast cancer, melanoma, etc.?

Checklist

Test results in the last six months, which can be brought to the doctor’s office

Imaging tests: CT scan of the head, MRI of the head, positron emission tomography (PET).

Those who have had surgery for meningioma also need to bring the surgery record and pathology results.

List of medications used

Medications used in the last 3 months, if available in boxes or packages, can be brought to the doctor’s office

Anti-tumor drugs: Temozolomide, Nitrosourea, Cisplatin, Bevacizumab, Gefitinib, etc.

Analgesic drugs: ibuprofen, codeine, aminophenol hydrocodone, etc.

Diagnosis

Diagnosis is based on

Medical history

Possible history of meningioma or surgery.

No history of tumors elsewhere in the body.

Clinical manifestations

Typical symptoms

Imaging findings of intracranial space, or symptoms such as seizures, headache, vomiting, limb dysmobility, and aphasia.

Physical signs

Physicians use physical examination to see if there are abnormalities in motor, ataxic, sensory, tendon, and pathological reflexes.

Motor function test: Observe whether the patient can complete movements such as lifting hands, sitting up, standing, walking, etc., and whether he/she needs assistance.

Ataxia examination: Observe the accuracy and speed with which the patient performs specific movements to assess whether ataxia exists. For example, the patient may use the pointer finger to point to the doctor’s fingertip and the tip of his or her nose, or slide the heel of one side of the foot from the knee to the foot along the front of the calf in the supine position.

Skin sensory examination: Slide a cotton swab over the patient’s skin or lightly prick the skin with a blunt needle to assess the degree of sensory impairment based on sensitivity to sensation.

Tendon reflex examination: Observe the contraction of the muscles of the upper arm and thigh when the tendons of the elbow and knee joints are tapped.

Pathological reflex examination: The doctor uses a blunt bamboo stick to gently stroke the patient’s sole and dorsum of the foot and other corresponding areas to see if the toe flexion and extension reactions are abnormal.

Cranial Magnetic Resonance Imaging (MRI) Scanning and Enhancement

Purpose of examination: To clarify the location, size and typology of the lesion, its effect on the surrounding tissues and the presence or absence of hydrocephalus, which is the main basis for the diagnosis of this disease.

Examination results: similar to benign meningioma, malignant meningioma.

Similar to benign meningiomas, tumors of malignant meningiomas also show significant enhancement, but the tumor margins are irregular, the borders are not obvious, and infiltration phenomena such as invasion of brain tissues, skull and scalp may occur [8-9].

Meningiomas that are larger in size and accompanied by edema and grow faster have a higher risk of malignancy

Note: Those who have metal implants such as dentures, plates in the body, or cardiac stents should inform the radiologist, and the doctor will decide whether the examination can be performed.

Cranial CT scan and enhancement

Purpose of examination: To determine whether there are intracranial space-occupying lesions and to observe whether there are lesions in the skull.

Examination results: malignant meningioma CT is usually low density, the lesion morphology is mostly irregular, and infiltrative destruction of bone can be seen.

Precautions: There is a certain amount of radiation, children, pregnant women are contraindicated.

Pathologic examination

The result of pathological examination is the “gold standard” of disease diagnosis.

It can clarify the typing and grading of the tumor, as well as the molecular characteristics of the tumor genes.

It has clear significance in determining the degree of malignancy of the tumor, short-term recurrence and prognosis of the tumor, postoperative radiotherapy, and even the selection of chemotherapy drugs.

Differential diagnosis

Malignant meningioma has little difference in performance with other intracranial tumors, and is mainly differentiated by imaging.

Benign meningioma

Most of them have homogeneous signal or density, smooth edges, clear tumor-brain interface, complete envelope, and obvious and uniform enhancement on enhancement scan.

Rarely appear cranial bone destruction.

Angioepithelial cell tumor

The tumor base is narrower, connected with the dura mater, lobulated, and grows faster.

The blood supply in the tumor body is richer, and flow-void signal shadow, necrosis, hemorrhage, and cystic degeneration can be seen.

It is poorly demarcated from brain tissue after enhancement.

Metastatic tumor

Most metastatic tumors have a history of primary tumor and rapid progression.

The enhancement is not uniform, or irregular nodular enhancement can be seen.

Treatment

Treatment objective: resection of lesions, improvement of symptoms, improvement of quality of life, prolongation of survival.

Treatment principle: Surgery is the main treatment, supplemented by radiotherapy.

Supportive treatment

Bed rest, close monitoring of consciousness, pupil, pulse, respiration and blood pressure.

When coma and respiratory difficulty occur, timely oxygen intake, tracheal intubation and ventilator-assisted ventilation can be performed.

Suspend eating and drinking if vomiting.

If there is swallowing disorder, nutritional support can be provided through nasal feeding tube or intravenous infusion route.

Keep the skin clean, turn over regularly, and use air cushion or soft cushion on the pressure-prone parts to prevent pressure sores.

Surgery

According to the pathological type and anatomical location of the tumor, the tumor should be removed through tumor resection, and the tumor should be completely cut as far as possible, and attention should be paid to the protection of neurological function at the same time. Non-special circumstances are suitable for surgical resection [9-10].

Surgical treatment is not recommended for those with widely expanding lesions, extensive involvement of nerves, bone, and internal carotid artery at the base of the skull, difficulty in removing the lesion by surgery, little benefit from surgery, or poor systemic condition that makes it difficult to tolerate.

Precautions

Postoperatively, different positions should be chosen according to different surgical methods: the basic principle is that the wound site should be facing upward, and the wound should be closely observed to see if there is any blood seepage.

When the patient is conscious, the head can be elevated 15-30 degrees as instructed by the doctor.

Postoperative observation of body temperature, respiration, heart rate, conscious state, changes in limb function, etc. is required.

CT examination is performed 6 hours after operation, and cranial MR review is performed at an early stage.

Radiotherapy

External radiation therapy (EBRT) and focal radiation therapy can be used as adjuvant treatment for malignant meningioma.

Precautions

The irradiated area should keep the skin clean and dry, avoid scratching, sun exposure and friction to prevent breakage and infection.

Drink more water and urinate more often during treatment.

Chemotherapy and targeted drugs

Except for hydroxyurea, which may have some effect on malignant meningioma, other chemotherapeutic drugs are basically ineffective for this disease.

The application of targeted drugs such as octreotide, paregoric acid and bevacizumab is also still in the research stage.

Symptomatic drug treatment

Dehydration drugs: Mannitol and glycerol fructose can be used to reduce edema and alleviate symptoms caused by peritumoral and postoperative edema.

Antiepileptic drugs: diazepam, carbamazepine, sodium valproate, etc. can be used to control seizures and avoid further aggravation of brain damage.

Neurotrophic therapy: If the symptoms of brain nerve damage are obvious, cytarabine, methylcobalamin, vitamin B12 and other drugs can be applied appropriately.

Analgesic drugs: If headache symptoms are obvious, ibuprofen, acetaminophen, aminophen hydrocodone and other drugs can be used.

Anti-infective drugs: If lung or urinary tract infection occurs, empirical antibiotics can be applied first, and relevant sensitive antibiotic treatment can be applied after the return of bacterial culture results.

Follow-up treatment

For patients with recurrence, comprehensive consideration should be made according to the site of recurrence, tumor size, intracranial pressure, and the patient’s basic condition.

If the tumor recurs locally, reoperation is usually recommended.

Generally malignant meningioma can be operated 3 times after recurrence.

Rehabilitation

When the vital signs are relatively stable, rehabilitation therapy can be carried out at an early stage to improve the quality of life.

The main contents include speech therapy, swallowing therapy, limb function training and so on.

Prognosis

Cure

The prognosis of this disease is poor, usually recurrence occurs 2-3 years after the first resection surgery, and the overall recurrence rate is as high as 80% [1-2].

The usual survival is 1.5 years, with a mortality rate of about 68% at 5 years and a 5-year survival of about 50%.

Prognostic factors

The prognosis is relatively good in the presence of the following [9-12]:

Site of the lesion: infiltration of cranial tissue with less invasion of nerves.

Extent of the lesion: the tumor is relatively limited in extent, with no infiltration of deep tissues.

Degree of tumor resection: total tumor resection or even extended resection is possible.

No major underlying disease.

Harmfulness

Hemiparesis, sensory impairment, and impaired consciousness lead to prolonged bed rest and serious deterioration of the patient’s quality of life.

Severe consciousness disorder can develop into vegetative state (vegetative).

Complications such as respiratory impairment, severe infections, and deep vein thrombosis can lead to death.

Seizures are prone to accidents such as falls, car accidents and burns, leading to fractures and traumatic brain injuries.

Severe and irreversible disability can bring great psychological disorder, cause mental illness, and increase the burden of family and society.

Everyday

Daily Management

Dietary management

Balanced nutrition, high protein, high calorie, high fiber diet (e.g. eggs, fish, poultry, fresh fruits and vegetables, etc.).

Eat a light diet, avoiding spicy and stimulating foods, foods that are easy to bloat, and foods that are too oily.

Supplement enough water to keep bowel movement smooth.

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

Patients with coma and swallowing disorders can be fed via nasal feeding tube.

Life management

Pay attention to rest, maintain a good daily routine, do not smoke, do not stay up late, do not overwork, etc..

After being discharged from the hospital, they should insist on home rehabilitation training and moderate exercise.

For patients who have been bedridden for a long time, they should pay attention to turn over and take care of themselves to avoid lung infection, urinary tract infection and bedsores.

Patients with limb weakness, blurred vision and seizures need to strengthen protective measures to prevent head and limb trauma and fracture injuries caused by falls.

Psychological support

Pay attention to your own mental health, release pressure in time, and avoid anxiety, depression and other bad emotions.

Family members should also pay attention to the patient’s mental state, listen patiently and interact with the patient more often.

If necessary, professional psychological counseling.

Disease monitoring

Monitor changes in symptoms such as headache, limb movement and sensory loss.

Monitor body temperature, skin and lower limb circumference for complications such as secondary infections, pressure sores and lower limb deep vein thrombosis.

Follow-up review

For patients with tumor resection, regular checkups should be conducted at 3 months, half a year and one year after surgery.

Follow-up mainly reviews the general condition, neurological function, cranial MRI examination and so on.

Prevention

The pathogenesis of this disease is not clear, and it is difficult to prevent it effectively, but it is possible to reduce the risk of the disease by the following measures:

Avoid unnecessary radiologic examinations and stay away from radiation sources.

People with family history of brain tumor should have regular physical examination.