Brain lymphoma is a rare but extremely dangerous disease. Elderly people and those with weakened immune systems are more likely to develop it. Unfortunately, to date, no drugs of this type have been invented for oncology, and patients can only count on delaying the inevitable death.

Causes of lymphoma

The reasons why lymphoma develops are divided into two categories. This is the influence of external factors and internal processes in the body. The environment plays a great role in the development of brain cancer.

Living or working in an area with increased radiation provokes the development of cancer in 97% of cases. A prerequisite for the appearance of the disease is also the influence of toxic substances such as vinyl chloride, a gas that is used in many industries, asparkam, used as a sugar substitute.

There is a theory that electromagnetic radiation from mobile phones and power lines can lead to lymphoma. Although this theory has not received scientific confirmation.

Internal factors that trigger the development of lymphoma are:

• undergoing radiation therapy to treat any disease;
• weakened immunity as a result of HIV infection;
• immunodeficiency after prevention of rejection of implanted donor organs.

Genetic predisposition plays an important role in the development of brain cancer. Medicine knows of cases where lymphoma affected first-degree relatives. In such patients, multiple benign tumors appear in their youth, but without proper treatment they can transform into cancer cells. More than 10% of children born to parents diagnosed with HIV are born with brain lymphoma.

Oncology can also appear against the background of mononucleosis, Epstein-Barr viral disease and chromosomal mutations. Recently, doctors have noted an increase in the number of patients with brain cancer. The reason for this was the deterioration of the general environmental situation in large cities and the nutritional characteristics of modern man. Increasingly, people began to eat large amounts of foods containing carcinogens.

Types of brain lymphomas

Brain cancer is classified into several types. Thus, it is easier to get a general picture of the disease and select the correct treatment.

1. Reticulosarcoma. Originates from reticular cells. In practice, it is extremely rare, so the history of its origin could not be fully ascertained. The disease is easily confused with lymphosarcoma. The symptoms of reticulosarcoma are multiple and depend on the location and stage of development. Initially appears in the lymph nodes. The disease is sensitive to radiation therapy. With early diagnosis and treatment, the patient has approximately 10 years of remission.


2. Microglioma. The most dangerous type of lymphoma, since its location does not always allow for full treatment. Malignant cancer cells grow very quickly and are almost incurable. Benign neoplasms spread more slowly and may not manifest themselves for quite a long time. Microglioma is diagnosed in half of patients with brain tumors. The disease arises from glial tissues at the cellular level. It is the primary type of formation that does not penetrate the bone cranial tissue and the inner lining of the brain. It looks like a dense clot of pinkish or red-gray color without clear boundaries. Can grow up to 15 cm in diameter. The disease can affect anyone, including children.
3. Diffuse histiocytic lymphoma. During the course of the disease, destruction occurs of both individual brain cells and its tissues. Metastases spread quite quickly, are diffuse in nature, and when they grow into healthy tissue, they give new impulses to the central nervous system. The patient feels hot, sweats profusely and rapidly loses weight. But, despite the fact that this type of oncology has an aggressive form, it is quite treatable.

Depending on the location of manifestation of lymphoma, single or multiple isolated lesions of brain tissue are divided. In 10% of cases, the eyes are affected with involvement of the meninges, extending to the spinal cord.

Symptoms of lymphoma

In brain lymphoma, the symptoms do not have any specific manifestations, which greatly complicates the diagnosis of the disease. Oncology can be suspected based on the following signs:

• increased intracranial pressure, causing constant headaches that do not go away after taking painkillers, intensify in the morning, in a supine position, when bending over. May be accompanied by nausea or vomiting;
• turning off certain functions of the part of the brain that the tumor is pressing on. This may be paralysis of the facial nerve, loss of speech, decreased vision, complete or partial loss of sensitivity on any side of the body, etc.;
• mental disorders. A sick person loses concentration, becomes absent-minded, and practically does not answer questions. There is an increased feeling of drowsiness, which can develop into lethargy. In some cases, a person may become rude, jokes become flat, the sense of self-criticism disappears, appetite increases to the point of gluttony;
• epileptic seizures. Periodically, convulsions occur with loss of consciousness or twitching of any muscle group. These symptoms occur with the following frequency: neurological deficit in 70% of cases, mental disorders in 43%, signs of intracranial hypertension in 33%, seizures in 14%. In patients with HIV, seizures occur in 25% of cases, and encephalopathy often develops at the age of 30-40 years.

In the later stages of lymphoma, personality changes occur. A person becomes emotionally unstable, his reactions are unpredictable, memory lapses appear.

Diagnosis of brain lymphoma

Identifying brain cancer is not so easy. Typically, a blood test does not show the presence of pathological changes in the body, and the symptoms are uninformative. If lymphoma is suspected, the doctor should conduct a complete examination of the patient. To begin with, a series of neurological tests are carried out to help identify disturbances in reflex function, coordination of movements, and the functioning of muscles and sensory organs. A full diagnosis is only possible with a complete clinical picture, but in this case there is practically no chance of a surgical cure. For early diagnosis of brain lymphoma, a number of instrumental and laboratory methods are used, including the following activities:

• Magnetic resonance therapy, which is carried out by injecting a contrast agent into the body through a vein. Brain lymphoma on MRI is visible on the computer due to the accumulation of this substance around the formation. which accumulates around the tumor;
• Tomography will show the presence of a tumor in the body;
• trephine biopsy - examination of biomaterial taken from brain tissue after opening the skull;
• stereotactic biopsy. This is an analysis of a biopsy, which is obtained by taking brain tissue through a hole made in the skull;
• electroencephalogram. It is used to detect the source of disturbances in the biopotential of the brain, their criticality and impact on the activity of the central nervous system as a whole;
• X-rays allow us to examine the presence of secondary signs of lymphoma and hypertension inside the brain;
• Ultrasonography is used to examine infants.

Tomography is one of the methods for diagnosing brain lymphoma

In addition to all of the above, the patient is prescribed a blood test and biochemistry. To determine primary brain lymphoma, radiography is performed, and for secondary lymphoma, ultrasound is performed.

Treatment options for brain cancer

Having diagnosed brain lymphoma, treatment is prescribed based on the stage of development of the disease, the location of the tumor and its size. It is not always possible to select the correct therapy due to the blood-brain barrier, which retains medications entering the body and neutralizes some of their components. There are three ways to stop the development of metastases, at least temporarily: chemotherapy, radiation therapy and surgery.

Chemotherapy

Brain lymphoma responds well to treatment with chemotherapy. Depending on the type of tumor and its sensitivity to different groups of drugs, mono or polychemotherapy may be prescribed. The drugs are administered to the patient by spinal puncture, because It is this approach that provides maximum effectiveness from drugs.

The choice of certain medications is determined only by the attending physician. Methotrexate is usually prescribed for a course of monochemotherapy. If it is necessary to use a combination of drugs, then preference is given to Cytarabine, Temozolomide or Etoposide.

Despite the fact that chemotherapy has good prerequisites for achieving remission, it is not without negative consequences. This happens because drugs act not only on diseased cells, but also on healthy ones. The occurrence of side effects depends on the drug chosen and its dosage. This can be severe nausea and dizziness, hair loss, dry mouth, weight loss, disruption of the gastrointestinal tract and a decrease in the body's protective functions. If chemotherapy has given positive results, and there is no secondary infection, then the patient can count on remission for several years.

Radiation therapy

Treatment by radiation is a proven and effective method, but not always in the case of brain lymphoma. If it is used, it is only in combination with chemotherapy or a bone marrow transplant operation. This decision is especially relevant for patients with a history of HIV.

Surgical intervention

Surgeries to remove tumors in the brain are practically not performed, because... this can lead to disruption of mental and nervous activity. Any attempts at radical intervention led to injuries to brain structures at a deep level.

Surgery is one of the treatment methods for brain lymphoma

The only type of surgery that is possible in the case of brain cancer is a stem cell transplant. But this procedure is performed only on young patients and does not guarantee a positive result.

Prognosis for lymphoma

For brain lymphoma, the prognosis for survival is not good. According to statistics, only 75% of patients receive remission for up to 5 years, but provided that the therapy was carried out on time. In old age, the figure drops to 40%. Possible relapses almost double the mortality rate among patients. Good results can be obtained if radical therapy is carried out and the tumor is not allowed to grow in a short period of time. In such cases, the lifespan can be extended to 10 years.

Source: oonkologii.ru

General concept of lymphomas of the central nervous system

The neoplasm is often a secondary lesion in stage 4 of various types of non-Hodgkin lymphoma. That is, initially growth is noted in the lymph nodes, then dissemination occurs with the formation of extranodal (extranodal) foci.

Primary central nervous system lymphoma (PCNSL) is a tumor arising from lymphoid tissue that originates in the brain or spinal cord and does not extend beyond them. To make such a diagnosis, a thorough examination and complete confidence in the absence of damage outside the nervous system are necessary.

Among primary localizations, PCNSL ranks second after gastric lymphomas. It is quite rare (in the structure of primary brain tumors its share is no more than 5%). Global incidence rates are 5-5.5 per 1 million population.

However, in recent decades there has been a significant increase in non-Hodgkin lymphomas, including PCNSL. Therefore, when carrying out a differential diagnosis of space-occupying formations of the central nervous system, this type of malignant formation should always be kept in mind.

Interest in this tumor is also due to the fact that it is extremely sensitive to chemotherapy and in 50% of cases complete remission can be achieved.

Morphologically, PCNSL is represented in 90% by diffuse large B-cell lymphoma. This is a highly malignant form.

At-risk groups

The main group is patients with immunodeficiency. PCNSL is diagnosed in 6-10% of HIV-positive individuals. On average, this complication occurs 5 years after the initial diagnosis of HIV infection. In almost 100% of cases, the appearance of lymphoma in these patients is associated with the Epstein-Barr virus. The average age of patients is 30-40 years, 90% of them are men.

The second group, in which PCNSL occurs much more often than in others (according to some data, 150 times), are persons receiving immunosuppressive therapy in connection with organ transplantation. The average age of patients is 55 years.

An increase in incidence is also observed in persons without immunodeficiency. However, in this group the age median is higher - over 60 years. Men are also more often affected (3:2 vs. women). The reasons for the growth are not reliably clear; doctors adhere to the viral theory.

Classification of CNS lymphomas

There is no stage gradation of PCNSL. The presence of one or more lesions, as well as damage to various parts of the central nervous system, does not affect the prognosis of the disease or the choice of treatment method.

Lymphomas are classified according to anatomical localization: single or multiple isolated lesions of brain tissue, lesions in the brain involving the eye (in 10% of cases), involving the meninges, spreading to the spinal cord, isolated lesions of the spinal cord, isolated lesions of the eye. In 85% of cases, primary brain lymphoma is located supratentorially, that is, in the hemispheres, and in 15% - infratentorially (cerebellum, ventricular region, brain stem). In the hemispheres, the frontal lobes are most often affected (20%).

What does CNS lymphoma look like?

According to localization, the tumor can manifest itself as single foci, varying in size, located in the hemispheres, in the basal ganglia, and the corpus callosum. In 35% of cases, multiple foci are observed (more often in people with immunodeficiency). Spinal cord lesions can occur primarily (70% in the lumbar region) or spread from the brain through the medulla oblongata by direct infiltration (cervical and thoracic regions), as well as dissemination of tumor cells into the cerebrospinal fluid.

Microscopically, lymphoma is a perivascular (around the vessels) accumulation of immunoblasts or centroblasts with infiltration of brain tissue.

Symptoms

Brain

This tumor does not have a specific symptom complex. Clinically, a brain mass can be suspected based on the following signs:

1. Increased intracranial pressure. It manifests itself as a bursting headache, which is not relieved by conventional painkillers, intensifies in the morning, in a lying position, when bending over, and is accompanied by nausea or vomiting.

2. Neurological deficit. This is a loss of certain functions due to the shutdown of the part of the brain on which the tumor puts pressure.

• Impaired movement of the limbs (with damage to the left frontal lobe - on the right, with right-sided localization - on the left). Movements are either completely absent (paralysis) or severely limited (paresis).
• Aphasia is a speech disorder.
• Double vision or loss of visual fields, a sharp decrease in vision.
• Swallowing problems (choking).
• Change in sensitivity of the right or left half of the body.
• Facial nerve paralysis.
• Hearing loss.
• Dizziness, unsteadiness when walking with damage to the cerebellum.

3. Mental disorders. Attention and concentration noticeably deteriorate, patients are inhibited and have difficulty answering questions. Drowsiness and even lethargy may develop. Signs of the frontal psyche: patients are sloppy, criticism decreases, a tendency to shallow jokes, rude, gluttonous, sexually disinhibited.

4. Seizures. Generalized convulsions with loss of consciousness or periodic twitching of any muscle group. These symptoms of brain lymphoma occur with the following frequency: neurological deficit in 70% of cases, mental disorders in 43%, signs of intracranial hypertension in 33%, seizures in 14%. In people with HIV infection, epileptic seizures are more common (25%), and a common symptom in them is the development of encephalopathy at a young age (30-40 years).

Spinal cord

Damage to this part of the central nervous system is manifested by signs of compression: impaired movement, sensitivity, loss of reflexes, urinary and fecal incontinence.

Diagnostics

Main

Lymphoma is not easy to identify. Although it conventionally refers to hematological diseases, there are usually no changes in the blood test. If the above symptoms are detected, the neurologist prescribes:

• Examination by an ophthalmologist.
• Electroencephalography.
• CT or MRI of the brain or spinal cord with and without contrast.

Lymphoma has certain signs that make it possible to suspect it already at the stage of neuroimaging. MRI shows single or multiple lesions that are either lower in density or do not differ from the surrounding brain tissue, usually homogeneous, sometimes ring-shaped. When contrast is introduced, they accumulate it intensively. Perifocal edema, calcifications, hemorrhages, and displacement of midline structures are observed less frequently than with other primary tumors of the central nervous system.

The diagnosis must be confirmed histologically. Stereotactic biopsy (STB) is the standard for suspected primary brain lymphoma. It is not recommended to prescribe steroids before the procedure, since due to their cytolytic effect they can significantly change the size and histological structure of the tumor. An open biopsy is performed only in cases where STB cannot be performed (for example, when localized in the brain stem). For spinal lymphoma, a laminectomy is performed to take a biopsy.

The resulting material is examined histologically, and immunohistochemical identification is also carried out (determination of the CD45 antigen).

Clarifying diagnostics

If the diagnosis of lymphoma is confirmed, the patient is examined further to:

• Search for extraneural lymphoma focus.
• Exclusion or confirmation of immunodeficiency (HIV).
• Assessment of general status to determine prognosis and future treatment.

Appointed:

• Detailed blood test.
• Biochemical analysis with determination of lactate dehydrogenase (LDH), creatinine clearance, albumin, urea, transaminases.
• Spinal puncture with general clinical and cytological examination of the cerebrospinal fluid.
• Determination of antibodies to HIV.
• Testing for antibodies to the Epstein-Bar virus, herpes, toxoplasmosis, syphilis, and viral hepatitis.
• CT scan of the chest.
• Ultrasound of lymph nodes.
• MRI of the abdominal cavity, pelvis.
• Fibrogastroscopy.
• Bone marrow puncture.
• In men - ultrasound of the testicles.
• If necessary, PET, spirometry, echocardiography. The general condition of the cancer patient is assessed using the Karnofsky scale (0-100%) or the ECOG scale (0-4 points).

Treatment

Surgical treatment for primary CNS lymphomas is usually not used. The exception is significant compression of the brain structures or spinal cord.

The main treatment method for such tumors is systemic chemotherapy and subsequent irradiation of the remaining lesions.

The most effective drug for PCNSL is methotrexate. It is used either alone or in combination with other cytostatics, most often with cytarabine. Clinical studies with rituximab also show good results.

For a full therapeutic effect, fairly high doses of methotrexate are required (up to 10 g/m2). But this drug is very toxic, it causes inhibition of hematopoiesis, impaired renal and liver function, and neuropathy. Therefore, it is important to choose a regimen that the patient can tolerate—therapeutic doses with high toxicity or palliative doses with low toxicity.

Methotrexate is administered by intravenous infusion only in a hospital setting. To reduce toxicity, it is necessary to additionally use calcium folinate (leucovorin) and administer large volumes of fluid. The course ranges from 4 to 8 cycles every 2 weeks.

After completing the chemotherapy course, radiation therapy is administered to the brain, including the eyes. The total dose is usually 30-36 Gy, the regimen is 2 Gy per session 5 times a week. If after chemotherapy, according to MRI, tumor foci persist, then additional local radiation exposure is prescribed.

In patients with primary spinal lymphoma, RT is considered the primary treatment option after surgical relief of spinal cord compression. However, due to the very rare occurrence of this localization of PCNSL, there is not enough data yet.

Forecast

The life expectancy of a patient with PCNSL without therapy is no more than 2 months. Timely diagnosis and the initiation of adequate complex treatment can completely cure 70% of patients. The prognosis for brain lymphoma is individual and depends on many factors. For PCNSL there is a special international prognostic index IELGS. Based on it, taking into account the patient’s risk factors, it is possible to predict overall 2-year survival.

Risk factors

1. Age over 60 years.
2. The patient's condition on the ECOG scale is more than 2 points (Karnofsky index >50%).
3. Increased plasma LDH.
4. Increased protein concentration in the liquor.
5. Damage to deep brain structures.

The prognosis for 2-year survival in the presence of 1 factor is 80%, 2-3 – 48%, and the determination of 4-5 factors reduces this figure to 15%.

Main conclusions

Primary CNS lymphoma is a fairly rare tumor. But it has its own characteristics that distinguish it from other malignant tumors of the nervous system.

Main characteristics of PCNS:

It occurs more often in people with immunodeficiency.

If lymphoma is suspected, steroids should not be given until a biopsy is obtained.

Unlike other brain tumors, it cannot be operated on.

It responds well to chemoradiation treatment.

Source: RosOnco.ru

Causes

There are two types of factors that provoke the development of pathology:

1. External negative influence;
2. Internal processes leading to the development of lymphoma.

Doctors recommend paying more attention to the influence of environmental factors on the brain. When a person lives in places with high levels of radiation, in 97 out of 100% problems in the head of an oncological nature are detected. The basis for the development of cancer is considered to be a substance – gas. Vinyl chloride is used in factories that make asparkam and a sugar substitute for diabetics.

There are statements that the development of a malignant tumor in the head occurs from electromagnetic radiation, as well as from harmful effects from telephones or high-voltage power lines. True, science has not yet been able to confirm the veracity of the assumptions.

When the cause of the appearance is established, you should carefully consider what can provoke the development of a tumor in the brain from the inside:

• Radiation exposure during radiation therapy.
• With HIV disease, the body's protective functions are significantly reduced. He is unable to fight the developing pathology.
• After organ transplant surgery. In this situation, the patient develops immunodeficiency.

Doctors do not rule out that heredity is one of the reasons for the appearance of degenerated brain cells. If the source of the disease was first-degree relatives, then the child showed a clinical picture even in his youth. However, at the first stage, the neoplasms are benign. When there is no treatment, the risk of cells transitioning from healthy to cancer increases.

Mononucleosis also causes an oncological tumor to develop inside the skull. Additional reasons:

• Epstein-Barr viral disease;
• Mutations in pairs of chromosomes.

Every day it is recorded that the number of people infected with the deadly disease is growing. A surge in diseases is often recorded in large cities. It's also worth paying attention to the food. In large retail outlets and on the market, it is less common to find a product that is grown naturally and ripened thanks to the sun, rather than a carcinogenic composition.

Symptoms

The danger of the disease lies in the absence of special signs of illness. Diagnosis is difficult because the patient does not complain of worsening of the condition.

To determine possible problems within the body, doctors recommend paying attention to each symptom described below.

Increased intracranial pressure

Intense headaches are provoked. The syndrome does not subside even after taking painkillers. In the morning, the headache becomes more intense. Lying down and bending over, the pain intensifies. Often additional symptoms are a gag reflex and nausea.

Loss of function

The patient loses certain functions controlled by the part of the brain where the tumor is located. As a result, an increase in the size of the tumor leads to pressure on the areas, and the patient loses skills.

Mental health disorders

The patient cannot concentrate, is often distracted, and cannot answer simple questions. The patient becomes sleepy, which can turn lethargic.

In other cases, a person is active, but may become rude when talking. He tries to joke, but these are flat, meaningless jokes. The patient stops criticizing himself. Appetite appears, leading to gluttony.

Epileptic seizures

The patient notes the appearance of convulsive phenomena, fainting, and twitching of a finger or hand is possible.

The frequency of manifestation of symptoms in this group: 70% – neurological deficit, 43% – mental disorders, 33% – intracranial pressure, 14% – convulsive phenomena. As a result of HIV infection, the patient's immunity decreases and then epilepsy attacks are observed in 25% of patients. Encephalopathy affects more than 50% of patients aged 30 to 40 years.

The final stages of lymphoma cause the patient's personality to change. There is instability in mood and emotions. It is impossible to predict a person's actions and reactions. The patient develops memory problems when there are no periods of memories.

Classification

Oncology of the brain organ is divided into three types. In order for treatment to be effective, the degree of damage to the human body and the source of abnormal cells must be clearly determined.

Let's look at the types of brain damage.

Reticulosarcoma

For certain reasons, connective tissue cells of the hematopoietic organs become malignant. Doctors rarely encounter this disease. Therefore, the pathology remains unexplored until the end. The clinical picture of the disease is similar to lymphosarcoma. These are always multiple foci of pathological development, depending on the location and degree of development of the disease.

Microglioma

Lymphoma classified as a dangerous type of pathology. The tumor is located where it is impossible to carry out full therapy. Diseased cells grow quickly, the volume of affected tissue increases. Does not respond to treatment. If a benign tumor has penetrated the brain, then the growth of the pathology is slow, without external manifestations.

Microglioma is found in 50% of patients with a tumor in the brain. The basis for growth is glial tissue. The tumor does not grow and does not affect the layers of the organ, does not grow into the bone tissue. A dense clot with fuzzy edges is visible on the screen. Cases have been recorded where the size of the tumor reached 15 centimeters. Microglioma develops in adults and children.

Lymphoma diffuse histiocytic

The disease destroys the brain from the inside. First, individual cells are destroyed, then tissues suffer. The development and spread of the tumor occurs rapidly. Metastases spread throughout the organ, affecting healthy tissue. The central nervous system receives new impulses from already damaged tissues. The patient's body temperature increases, sweating increases, and body weight decreases. This type of cancer, quickly spreading throughout the body, is sensitive to the treatment.

Lymphoma of the central nervous system and brain can form a single focus of pathological development and a multiplicity of foci. In 10 patients out of 100 suffering from this type of cancer, the eyes, the membranes of the organ in the skull, and the spinal cord are affected.

In the vast majority of cases of lymphoma, the tumor spreads within the cerebral hemispheres (85%). Cerebellar involvement may occur in 15% of cases. The same number of patients have tumors in the ventricles of the brain and in the brainstem.

Diagnostics

It has already been said that diagnosis of the disease is carried out only if you consult a doctor for another disease. A blood test is not considered a reliable source of tumor determination, so a comprehensive examination prescribed by a doctor is needed.

The following medical equipment is used to carry out the procedure:

• MRI. The patient is first injected with contrast into a vein. On an MRI, the lymphoma will immediately appear, surrounded on all sides by a contrast agent.
• Tomography. Here, the study will confirm that there is a tumor and warn about the need for treatment.
• Trephine biopsy. This is a study of part of the biological material taken from the site of the lesion after opening the skull.
• Stereotactic biopsy. Here, the resulting biomaterial occurs through an opening in the bones of the skull.
• Electroencephalogram. This method can be used when the source of the pathology is identified. The influence and criticality of the situation with the Central nervous system is measured.
• X-ray. The photo shows a secondary sign of oncology and intracranial pressure.
• The study is carried out in children using ultrasonography.

Treatment

The doctor, having received research data confirming the diagnosis of lymphoma, prescribes treatment on an individual basis. Three ways to fight:

• Chemical therapy;
• Radiation exposure;
• Operation.

Chemotherapy

An effective way to fight cancer. The oncologist selects medications individually and calculates the dose. Using several medications at the same time gives greater results.

Chemical therapy and radiation are often combined. Preparations containing chemicals:

• Cytarabine;
• Etoposide;
• Methotrexate;
• Cyclophosphamide;
• Chlorambucil, etc.

For treatment, medications with monoclonal antibodies are used. The disadvantage of using chemicals to attempt recovery is that it destroys diseased and healthy cells at the same time.

Side effects after undergoing chemotherapy:

• Anemia develops, leading to weakness in the body and muscles.
• Vomiting, nausea.
• Digestive system disorder.
• Loss of hair.
• Constant feeling of dryness. In this case, small ulcers and wounds on the mucous membrane appear in the oral cavity.
• Body weight is rapidly decreasing.
• The body's protective shell does not work. This means that third-party infections freely penetrate into the body.

If you need pain relief, take Celebrex.

Radiation exposure

Since chemotherapy does not always give positive results in the treatment of oncology, radiation exposure becomes an additional means that enhances the effect of the first. Radiation irradiation reaches the metastases, destroying the source of excretion. It is not used as an independent way to fight cancer.

Lymphoma is a tumor arising from lymphoid tissue. This is, in fact, a malignant blood disease, the primary location of which is outside the bone marrow. There are about 80 types of lymphomas. For a long time, there was a classification dividing these tumors into two large groups: Hodgkin lymphoma and non-Hodgkin lymphoma. The main morphological criterion of difference is the presence of special Berezovsky-Sternberg cells in the tumor structure in Hodgkin lymphomas. Since 2001, a different classification has been used: these tumors are divided into groups depending on the cells from which they develop (precursors of T- or B-lymphocytes or their mature forms).

Lymphoid tissue is located everywhere in the body. Therefore, its uncontrolled growth can occur in any organ. But, of course, the main accumulation of lymphocytes is the lymph nodes. Two thirds of such neoplasms primarily arise in them - this is the nodal form. The rest are extranodal, localized in the spleen, skin, stomach, intestines, brain, and other organs.

General concept of lymphomas of the central nervous system

The neoplasm is often a secondary lesion in stage 4 of various types of non-Hodgkin lymphoma. That is, initially growth is noted in the lymph nodes, then dissemination occurs with the formation of extranodal (extranodal) foci.

Primary central nervous system lymphoma (PCNSL) is a tumor arising from lymphoid tissue that originates in the brain or spinal cord and does not extend beyond them. To make such a diagnosis, a thorough examination and complete confidence in the absence of damage outside the nervous system are necessary.

Among primary localizations, PCNSL ranks second after gastric lymphomas. It is quite rare (in the structure of primary brain tumors its share is no more than 5%). Global incidence rates are 5-5.5 per 1 million population.

However, in recent decades there has been a significant increase in non-Hodgkin lymphomas, including PCNSL. Therefore, when carrying out a differential diagnosis of space-occupying formations of the central nervous system, this type of malignant formation should always be kept in mind.

Interest in this tumor is also due to the fact that it is extremely sensitive to chemotherapy and in 50% of cases complete remission can be achieved.

Morphologically, PCNSL is represented in 90% by diffuse large B-cell lymphoma. This is a highly malignant form.

At-risk groups

The main group is patients with immunodeficiency. PCNSL is diagnosed in 6-10% of HIV-positive individuals. On average, this complication occurs 5 years after the initial diagnosis of HIV infection. In almost 100% of cases, the appearance of lymphoma in these patients is associated with the Epstein-Barr virus. The average age of patients is 30-40 years, 90% of them are men.

The second group, in which PCNSL occurs much more often than in others (according to some data, 150 times), are persons receiving immunosuppressive therapy in connection with organ transplantation. The average age of patients is 55 years.

An increase in incidence is also observed in persons without immunodeficiency. However, in this group the age median is higher - over 60 years. Men are also more often affected (3:2 vs. women). The reasons for the growth are not reliably clear; doctors adhere to the viral theory.

Classification of CNS lymphomas

There is no stage gradation of PCNSL. The presence of one or more lesions, as well as damage to various parts of the central nervous system, does not affect the prognosis of the disease or the choice of treatment method.

Lymphomas are classified according to anatomical localization: single or multiple isolated lesions of brain tissue, lesions in the brain involving the eye (in 10% of cases), involving the meninges, spreading to the spinal cord, isolated lesions of the spinal cord, isolated lesions of the eye. In 85% of cases, primary brain lymphoma is located supratentorially, that is, in the hemispheres, and in 15% - infratentorially (cerebellum, ventricular region, brain stem). In the hemispheres, the frontal lobes are most often affected (20%).

What does CNS lymphoma look like?

According to localization, the tumor can manifest itself as single foci, varying in size, located in the hemispheres, in the basal ganglia, and the corpus callosum. In 35% of cases, multiple foci are observed (more often in people with immunodeficiency). Spinal cord lesions can occur primarily (70% in the lumbar region) or spread from the brain through the medulla oblongata by direct infiltration (cervical and thoracic regions), as well as dissemination of tumor cells into the cerebrospinal fluid.

Microscopically, lymphoma is a perivascular (around the vessels) accumulation of immunoblasts or centroblasts with infiltration of brain tissue.

Symptoms

Brain

This tumor does not have a specific symptom complex. Clinically, a brain mass can be suspected based on the following signs:

1. Increased intracranial pressure. It manifests itself as a bursting headache, which is not relieved by conventional painkillers, intensifies in the morning, in a lying position, when bending over, and is accompanied by nausea or vomiting.

2. Neurological deficit. This is a loss of certain functions due to the shutdown of the part of the brain on which the tumor puts pressure.

• Impaired movement of the limbs (with damage to the left frontal lobe - on the right, with right-sided localization - on the left). Movements are either completely absent (paralysis) or severely limited (paresis).
• Aphasia is a speech disorder.
• Double vision or loss of visual fields, a sharp decrease in vision.
• Swallowing problems (choking).
• Change in sensitivity of the right or left half of the body.
• Facial nerve paralysis.
• Hearing loss.
• Dizziness, unsteadiness when walking with damage to the cerebellum.

3. Mental disorders. Attention and concentration noticeably deteriorate, patients are inhibited and have difficulty answering questions. Drowsiness and even lethargy may develop. Signs of the frontal psyche: patients are sloppy, criticism decreases, a tendency to shallow jokes, rude, gluttonous, sexually disinhibited.

4. Seizures. Generalized convulsions with loss of consciousness or periodic twitching of any muscle group. These symptoms of brain lymphoma occur with the following frequency: neurological deficit in 70% of cases, mental disorders in 43%, signs of intracranial hypertension in 33%, seizures in 14%. In people with HIV infection, epileptic seizures are more common (25%), and a common symptom in them is the development of encephalopathy at a young age (30-40 years).

Spinal cord

Damage to this part of the central nervous system is manifested by signs of compression: impaired movement, sensitivity, loss of reflexes, urinary and fecal incontinence.

Diagnostics

Main

Lymphoma is not easy to identify. Although it conventionally refers to hematological diseases, there are usually no changes in the blood test. If the above symptoms are detected, the neurologist prescribes:

• Examination by an ophthalmologist.
• Electroencephalography.
• CT or MRI of the brain or spinal cord with and without contrast.

Lymphoma has certain signs that make it possible to suspect it already at the stage of neuroimaging. MRI shows single or multiple lesions that are either lower in density or do not differ from the surrounding brain tissue, usually homogeneous, sometimes ring-shaped. When contrast is introduced, they accumulate it intensively. Perifocal edema, calcifications, hemorrhages, and displacement of midline structures are observed less frequently than with other primary tumors of the central nervous system.

The diagnosis must be confirmed histologically. Stereotactic biopsy (STB) is the standard for suspected primary brain lymphoma. It is not recommended to prescribe steroids before the procedure, since due to their cytolytic effect they can significantly change the size and histological structure of the tumor. An open biopsy is performed only in cases where STB cannot be performed (for example, when localized in the brain stem). For spinal lymphoma, a laminectomy is performed to take a biopsy.

The resulting material is examined histologically, and immunohistochemical identification is also carried out (determination of the CD45 antigen).

Clarifying diagnostics

If the diagnosis of lymphoma is confirmed, the patient is examined further to:

• Search for extraneural lymphoma focus.
• Exclusion or confirmation of immunodeficiency (HIV).
• Assessment of general status to determine prognosis and future treatment.

Appointed:

• Detailed blood test.
• Biochemical analysis with determination of lactate dehydrogenase (LDH), creatinine clearance, albumin, urea, transaminases.
• Spinal puncture with general clinical and cytological examination of the cerebrospinal fluid.
• Determination of antibodies to HIV.
• Testing for antibodies to the Epstein-Bar virus, herpes, toxoplasmosis, syphilis, and viral hepatitis.
• CT scan of the chest.
• Ultrasound of lymph nodes.
• MRI of the abdominal cavity, pelvis.
• Fibrogastroscopy.
• Bone marrow puncture.
• In men - ultrasound of the testicles.
• If necessary, PET, spirometry, echocardiography. The general condition of the cancer patient is assessed using the Karnofsky scale (0-100%) or the ECOG scale (0-4 points).

Treatment

Surgical treatment for primary CNS lymphomas is usually not used. The exception is significant compression of the brain structures or spinal cord.

The main treatment method for such tumors is systemic chemotherapy and subsequent irradiation of the remaining lesions.

The most effective drug for PCNSL is methotrexate. It is used either alone or in combination with other cytostatics, most often with cytarabine. Clinical studies with rituximab also show good results.

For a full therapeutic effect, fairly high doses of methotrexate are required (up to 10 g/m2). But this drug is very toxic, it causes inhibition of hematopoiesis, impaired renal and liver function, and neuropathy. Therefore, it is important to choose a regimen that the patient can tolerate—therapeutic doses with high toxicity or palliative doses with low toxicity.

Methotrexate is administered by intravenous infusion only in a hospital setting. To reduce toxicity, it is necessary to additionally use calcium folinate (leucovorin) and administer large volumes of fluid. The course ranges from 4 to 8 cycles every 2 weeks.

After completing the chemotherapy course, radiation therapy is administered to the brain, including the eyes. The total dose is usually 30-36 Gy, the regimen is 2 Gy per session 5 times a week. If after chemotherapy, according to MRI, tumor foci persist, then additional local radiation exposure is prescribed.

In patients with primary spinal lymphoma, RT is considered the primary treatment option after surgical relief of spinal cord compression. However, due to the very rare occurrence of this localization of PCNSL, there is not enough data yet.

Forecast

The life expectancy of a patient with PCNSL without therapy is no more than 2 months. Timely diagnosis and the initiation of adequate complex treatment can completely cure 70% of patients. The prognosis for brain lymphoma is individual and depends on many factors. For PCNSL there is a special international prognostic index IELGS. Based on it, taking into account the patient’s risk factors, it is possible to predict overall 2-year survival.

Risk factors

1. Age over 60 years.
2. The patient's condition on the ECOG scale is more than 2 points (Karnofsky index >50%).
3. Increased plasma LDH.
4. Increased protein concentration in the liquor.
5. Damage to deep brain structures.

The prognosis for 2-year survival in the presence of 1 factor is 80%, 2-3 – 48%, and the determination of 4-5 factors reduces this figure to 15%.

Main conclusions

Primary CNS lymphoma is a fairly rare tumor. But it has its own characteristics that distinguish it from other malignant tumors of the nervous system.

Main characteristics of PCNS:

It occurs more often in people with immunodeficiency.

If lymphoma is suspected, steroids should not be given until a biopsy is obtained.

Unlike other brain tumors, it cannot be operated on.

It responds well to chemoradiation treatment.

A pathological change in lymphoid tissue in lymph nodes and organs within the body, characterized by an uncontrolled accumulation of altered lymphocytes, is designated by the term “lymphoma”. Brain lymphoma is a tumor that is malignant. It occurs in the process of uncontrolled division of pathological cells.

Lymphoma is a malignant brain tumor

This is a new formation consisting of white blood cells: malignant or benign. Tumor tissue is constantly growing. If the node grows slowly, compresses nearby tissues, but does not penetrate into them and does not metastasize, and its cells are identical, the tumor is considered benign.

A small node can remain in the brain for a long time and not cause much harm to the patient. The tumor can be removed if it has not grown and is considered surgical. When it degenerates into a malignant one, the tumor begins to grow quickly and uncontrollably, penetrate into tissues, grow inside the brain matter, thereby posing a threat to human life.

What types of brain tumors are there?

Brain cancer is a malignant growth inside the skull that can be primary or secondary. Brain lymphoma is formed from brain tissue and its membranes; it grows, compresses the tissue, but does not metastasize, and is called primary.

Secondary, brain lymphoma arises from malignant B-cell non-Hodgkin lymphomas that spread from other organs through the bloodstream. Primary localized lymphoma is easier to excise and treat. Secondary tumors are more difficult to operate and treat, since it is necessary to find and remove the main tumor in other areas of the body.

Conclusion! Tumors can be benign and malignant, primary and secondary origin, operable or inoperable.

Causes of brain lymphoma

According to statistics, before the advent of HIV and AIDS, brain lymphomas occurred in only 3% of cases of formations growing in the brain and changing it. Although lymphoma cells belong to the group of B-lymphocytes, it is classified as a brain tumor due to its characteristic clinical picture and treatment features.

A brain tumor has increased neurotoxicity, and its treatment is complicated by the blood-brain barrier. Primary brain cancer is diffuse B-cell formations; low-grade and T-cell tumors and Burkitt's lymphoma are less common.

Reduced immunity is a serious factor in the development of lymphomas. In this regard, symptoms of brain lymphoma may appear after:

• heart or other organ transplants;
• diseases of HIV or infectious mononucleosis;
• high doses of radiation (97%);
• contact with carcinogens;
• mutations of DNA chromosomes, which is associated with a hereditary predisposition. If we talk about the external environment, brain cancer can arise from prolonged contact with vinyl chloride in production where plastics are used. Risk factors for the disease include the artificial sugar substitute - Asparkam, radiation from a mobile phone, computer, and the negative impact of high-voltage lines.

Symptoms of brain lymphoma

A brain tumor may not appear for a long time, or its symptoms may confuse even experienced specialists who make mistakes in diagnosis. Signs of lymphoma are monitored by changes in neurological characteristics, since it develops naturally according to the principles of oncology.

It is easier to detect brain tumors that develop close to the surface of the brain matter. The ability to promptly begin treatment of important brain structures in the early manifestations of a tumor is very important, since when intracranial pressure increases, attacks in the form of migraines, nausea, and vomiting can begin. Patients complain of blurred vision and double vision.

The following symptoms of brain lymphoma can be recognized by abnormal conditions as:

• speech and vision are impaired;
• lability of emotions occurs, the patient is prone to epileptic seizures, neuropathies, and mental disorders;
• hydrocephalus (dropsy) is determined;
• neuropathies, mental disorders;
• Patients' consciousness is confused, they suffer from hallucinations;
• motor coordination is lost;
• limbs go numb;
• headaches with fever and general weakness;
• loss of appetite and kilograms of accumulation in the subcutaneous tissue.

Brain tumors in children and adolescents manifest themselves:

• intracranial hypertension;
• attacks of epilepsy;
• meningeal signs;
• cognitive impairment;
• damage to the nerves under the skull;
• neurological signs in the lesions (hemiparesis, ataxia, aphasia, visual impairment), which depends on the location of development and size of perifocal edema.

If there are lesions in deep areas, the patient’s behavioral and personal qualities change.

Metastases in brain cancer form a secondary formation, which is manifested not only by excruciating pain inside the head, deterioration of hearing and visual abilities, symptoms of intoxication and swelling of the optic nerve, but also ischemic cerebral infarction and hemorrhage. Hematoma or blood clots lead to disruption of brain activity, which is why encephalopathy develops.

Classification of brain lymphomas

According to the classification, primary lymphoma has four clinical variants:

• intracerebral nodes: single or multiple;
• diffuse infiltration: meningeal or periventricular;
• infiltration of the retina or vitreous body (appears before or after a meningeal or parenchymal tumor);
• spinal cord lymphoma.

The patient’s physical condition is assessed in accordance with the ECOG (Eastern Cooperative Oncology Group) scale:

• 0 – no restrictions for normal physical activity;
• 1 – physical activity is limited: the patient performs simple work at home or in the office, undergoes outpatient treatment;
• 2 – the patient can take care of himself, but cannot engage in work, spends 50% of the time in bed;
• 3 – the patient can only partially take care of himself, sits or lies in bed 60-70% of the time;
• 4 – the patient is completely disabled, cannot take care of himself, cannot walk, sit or lie;
• 5 – death.

The classification of brain tumors combines B-cell and T-cell lymphomas. B-cell lymphomas include the following types of brain lymphomas:

• diffuse large cell lymphoma, 30% of cancer patients suffer from this type. Lymphoma is treatable, 5-year prognosis is positive in 50%;
• small cell lymphocytic lymphoma, characterized by high malignancy and slow growth. B-cell formations account for 7% and degenerate into a fast-growing tumor;
• follicular lymphoma, grows slowly with low malignancy, diagnosed in 22% of all lymphomas, prognosis - more than 60% of patients cross the 5-year survival threshold;
• lymphoma from tumor cells of the mantle zone, occurs in 6% of cases, with slow growth, it is characterized by malignancy and an unfavorable prognosis: less than 20% of cancer patients survive. The main formation - Burkitt's lymphoma occurs in 90% of men after 30 years of age, but it is detected in 2% of all cases of lymphoma.

Of T-tumors, the classification includes:

• T-lymphoblastic malignant lymphoma of the brain. Boys aged 18-20 years old are most often affected (75%). In the later stages, and when the spinal cord is involved in the oncological process, the chance of survival in patients is small, only about 20%. If there are no metastases in the spinal cord, the prognosis will be more optimal;
• anaplastic large cell lymphoma of the brain. It is diagnosed in working age men and women equally. Treatment of a brain tumor with chemotherapy produces positive results due to the susceptibility of the lymphoma to the drugs;
• extranodal T-cell lymphoma of the brain, affecting people of all ages. Survival depends on the stage of the cancer process and adequate treatment.

Primary tumors also include the following types of brain tumors, their names coincide with the name of the tissue from which they were formed:

• acoustic neuroma (schwannoma);
• astrocytoma or glioma, which includes anaplastic astrocytoma and glioblastoma;
• ependymoma;
• epidimoblastoma;
• meduloblastoma;
• meningioma;
• neuroblastoma;
• oligodendroglioma;
• pineoblastoma.

Informative video

Diagnosis of the disease

How to identify a brain tumor?

If symptoms appear, you should consult a physician to evaluate your health and test results. First of all, the activity of tendon reflexes, tactile and pain sensitivity is checked. If a tumor in the brain is suspected, the diagnosis is confirmed by the following methods:

• computer and magnetic resonance imaging;
• biopsy of brain tissue;
• spinal cord puncture: examine cells, protein, viral load, flow cytometry;
• blood testing, including tumor markers;
• X-ray examination.

In special cases, the following is prescribed:

• trephine biopsy: open the skull and examine the brain tissue;
• stereotactic biopsy: a small hole is made in the skull and brain tissue is taken for analysis.

Secondary extranodal lymphomas are difficult to diagnose because there is no enlargement of the lymph nodes, because The primary tumor does not form in the lymph nodes, but in other organs, such as the lungs, intestines, spleen or stomach. The extranodal form also occurs in primary brain lymphomas: non-Hodgkin, usually B-cell.

The difficulty of diagnosis is that at the first stage there are no specific symptoms; a peripheral blood test and bone marrow biopsy may turn out to be normal.

Early stages of brain cancer can be suspected based on patient complaints of weakness, sweating, digestive disorders, weight loss, and fever. General cerebral and/or focal neurological symptoms are possible.

It is mandatory to differentially diagnose primary CNS lymphoma in order to exclude:

• other neoplasms;
• herpetic encephalitis;
• multiple sclerosis;
• pseudotumor cerebri;
• intracerebral hemorrhage;
• toxoplasmic encephalitis due to HIV infection;
• metastases in brain tissue.

Treatment of brain lymphoma

Treatment of brain lymphomas is not always effective and remission is not always long-lasting. This happens due to the blood-brain barrier: incoming drugs are delayed and some components of these drugs are neutralized. This is where the difficulty in selecting adequate therapy arises.

Brain treatment begins with the prescription of corticosteroids to eliminate swelling and improve the patient’s well-being. For chemotherapy, large doses of Methotrexate are most often used for injection into a vein or spinal cord. For combined chemistry, certain doses of Cytarabine, Rituximab, and Temozolomide are used simultaneously. For long-term remission, a course of radiation (radiotherapy) is added to chemotherapy.

Symptomatic therapy is also used to treat concomitant disorders such as hypertension, pain, neuropathy, hypercalcemia.

Surgical therapy

Tumor excision is rarely performed because they are most often inoperable. The irradiation method is used more often, it improves the condition of patients, but remission does not last long - 6-10 months, especially for HIV, and for AIDS - only 4-6 months.

The most modern effective and minimally invasive method is radiosurgical gamma knife surgery. There is no need to open the skull. Gamma Knife is a device consisting of a special helmet with built-in radioactive radiation. The helmet is placed on the patient's head.

The rays coming from the emitters are brought together to one point. It is specially calculated based on the location of the tumor. The formation is destroyed by point radio irradiation. It penetrates into the most inaccessible places, so minimal irradiation of surrounding healthy tissue is possible.

Prognosis for brain lymphoma

Without treatment, patients live 2-3 months. After chemotherapy, patients live 4 or more years. The five-year life threshold is approximately 40%. Life expectancy increases after stem cell transplantation. Young people live longer than older people. After irradiation and chemotherapy, there are complications: headache, impaired consciousness, tissue necrosis.

Informative video

Primary brain lymphoma is a fairly rare disease caused by damage to lymphoid tissue in the brain of the central nervous system. The pathology is also known under other names - reticulosarcoma, microglioma, diffuse histocytic lymphoma.

What it is

Brain lymphomas are non-Hodgkin B-cell tumors characterized by a high degree of malignancy. Neoplasms of this type grow directly from brain tissue, soft membranes of the brain (less commonly, the eyeball). In most cases, the primary tumor remains within the central nervous system, rarely metastasizing.

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Like any oncological tumor, lymphoma develops gradually and in the first stages rarely gives pronounced specific symptoms. For this reason, detection of lymphoma does not always occur at a stage when an effective and rapid cure for the disease is possible.

In addition, the presence of the blood-brain barrier (a physiological barrier that protects the brain from toxins and other pathogenic factors) prevents the full use of typical treatment methods for lymphomas. For this reason, treatment of this disease is difficult and often has a poor prognosis.

Causes

Most often, lymphomas occur in people over 50-60 years of age with a weakened immune system.

The reasons for weakened immunity and the development of lymphomas can be the following:

• organ transplantation (heart, kidney and liver);
• presence of immunodeficiency virus;
• the presence of the Epstein-Barr virus and mononucleosis caused by this virus;
• exposure to high doses of radiation;
• contact with carcinogenic substances;
• predisposition to chromosomal mutations, inherited.

Brain lymphoma in HIV is a kind of complication caused by a malfunction of the lymphatic system. In such patients, treatment of the disease is difficult due to the limited possibility of using strong drugs and is often fraught with death.

Recently, doctors all over the world have noted a trend towards an increase in the number of malignant diseases associated with lesions of lymphoid tissue. Perhaps the reason is the deterioration of the general environmental situation in large cities, as well as the dietary habits of modern people, who consume an increased amount of foods with chemical additives - potential carcinogens.

In addition, the number of HIV-infected patients developing brain lymphoma is increasing.

Symptoms of brain lymphoma

In the case of non-Hodgkin's lymphoma of the brain, neoplasms may originate from lymphoid tissue of initially non-lymphoid organs. In addition, the disease can be secondary - in this case, the brain is involved in a process that began elsewhere in the body. Secondary cancer foci (metastases) are single or multiple tumor nodes.

The following forms of brain lymphoma are also found:

• diffuse meningeal infiltration (the so-called leptomeningeal form, affecting primarily the meninges);
• ocular lymphoma (orbital) – a neoplasm that affects the vitreous body of the organ of vision or the retina;
• spinal form, affecting not only the brain, but also the spinal cord.

Clinical symptoms of brain lymphoma resemble other cancers of the central nervous system.

The list of manifestations includes:

• headache;
• drowsiness;
• focal neurological symptoms (features of this group of signs depend on the location of the primary lesion and its size);
• epileptic seizures;
• emotional disturbances;
• neuropathy;
• hydrocephalus (accumulation of excess cerebrospinal fluid in the subarachnoid space and ventricles of the brain);
• speech disorders;
• visual disturbances;
• hallucinations;
• loss of coordination of movements;
• weakness and numbness in the hands.

At later stages, as the malignant process progresses and the tumor grows, signs of personality changes, inappropriate emotional reactions, and memory loss may be observed. These disorders are especially characteristic of lesions of the frontal and temporal lobes.

Diagnostics

If primary brain lymphoma is suspected, magnetic resonance imaging (MRI) is prescribed. This imaging method allows you to study in detail the condition of the brain, membranes and internal cavities inside the skull. If necessary (to check the condition of the vessels), tomography with a contrast agent is prescribed.

Clarifying the diagnosis may require additional research:

• lumbar puncture and further examination of the cerebrospinal fluid for the presence of specific cells (cancer markers);
• stereotactic biopsy: a sample of tumor tissue - a biopsy - is taken through a small hole in the skull and then examined in the laboratory (this method allows us to identify the degree of malignancy of the tumor, the stage of its development and prescribe adequate therapy);
• trephine biopsy - taking a sample by fully opening the skull;
• blood analysis.

If a secondary nature of brain lymphoma is suspected, additional studies of the body are prescribed - ultrasound, CT, radiography.

Sometimes a bone marrow biopsy may be necessary (if the primary lesion is in the bone marrow). This variant of the development of the disease is represented by infiltration of the brain parenchyma with leukocytes.

Secondary brain lesions cause similar symptoms with a predominance of pain symptoms. Patients are plagued by a painful bursting headache, nausea and vomiting, swelling of the optic nerves, deterioration of vision and hearing.

In a number of clinical cases, secondary brain lesions cause sudden hemorrhages and ischemic cerebral infarctions. Subdural (epidural and intracerebral) hematomas disrupt brain activity and lead to progressive encephalopathy.

You can find out the cost of treatment for lymphoma in Moscow.

Treatment

Over the past decades, brain radiotherapy has remained a priority in the treatment of lymphomas. This technique is indeed quite effective, but the positive dynamics after exposure to radiation are temporary.

More durable results are achieved when systemic chemotherapy is used in conjunction with radiation. Patients with adequate immune status (doctors call such patients “immunocompetent”) respond well to complex treatment: the period of remission is extended to several years.

Immunotherapy and targeted therapy can be used as experimental methods, although long-acting drugs against lymphomas have not yet been created.

Symptomatic therapy is also used - treatment of concomitant disorders:

• hypertension;
• pain syndrome;
• neuropathy;
• hypercalcemia.

Palliative treatment at the last stage of malignant cerebral lymphomas involves relief of intense pain symptoms with narcotic analgesics. Often this is the only help that doctors can provide to patients. Surgical removal of brain lymphomas is impossible in almost 100% of cases, since the risk of disruption of the patient’s nervous and mental activity is too great.

In the past, attempts at radical treatment of brain tumors were made, and repeatedly, but in most cases such therapy ended in damage to the deep structures of the brain. The difficulty also lies in the inability to determine clear boundaries of lymphomas.

Video: About brain tumors

Forecast

When brain lymphomas occur in patients with AIDS, the prognosis is unfavorable. The life expectancy of patients does not exceed several months. Primary lymphomas in people with normal immunity who are exposed to radiation therapy may stop growing and developing for some time, but this period does not exceed 12-18 months.

The use of complex therapy improves the prognosis. Exposure to chemotherapy increases the life expectancy of patients by several years. However, complete cure of brain lymphoma is extremely rare.

Lymphoma is a pathological malignant neoplasm consisting of atypical lymphocytes that actively multiply and form a tumor. This formation affects lymphoid tissue and can occur in different organs. Primary brain lymphoma is a fairly rare disease, diagnosed in five people per million of the population. Among all primary brain formations, lymphoma accounts for 1-3% of all cases.

Classification

There is non-Hodgkin's lymphoma and Hodgkin's disease. The first option is characterized by the appearance of a tumor due to a mutation of one lymphocyte cell and can affect the brain. Hodgkin's lymphoma affects the entire lymphatic system and is named after the scientist who first described the pathology.

Non-Hodgkin's lesions can be primary or secondary, most often affecting men. Primary tumors in the brain are extremely rare; more often a tumor arises due to metastasis from another location and is called secondary.

According to the WHO classification, the following types of B tumors are distinguished:

T-tumors include:

Mediastinal lymphomas

Mediastinal cancer occurs mainly in adults aged 20-40 years. The incidence is 3-7% of all types of neoplasms with different localization and malignancy. For the most part, it is not cancer that occurs in the mediastinum, but benign tumors, in a ratio of 80 to 20. Regarding lymphomas, they are formed in the lymphatic system of the mediastinum. The main signs that you should pay attention to are an increase in lymphatic
nodes in the neck or axillary sinuses. They may not be painful at first, but later discomfort, pain and redness appear in the area of ​​the mediastinal lesion. Also, mediastinal cancer has such a feature as breathing complications, dysphagia or shortness of breath. Wheezing or coughing may occur. All this is associated with the growth of lymphoma in the mediastinal cavity and stopping the normal functioning of the respiratory system.

As a rule, there is no point in using a surgical method, since atypical cells are carried with the lymph flow to different organs, which is a particular difficulty in the treatment of lymphomas. As in the case of the brain, the main method remains a course of radiation and chemotherapy. Often the formation of lymphoma in the mediastinum becomes the cause of a secondary tumor in the brain, as a result of metastasis.

Additional methods include bone marrow transplantation; the method is innovative, but expensive and specific. As well as immunotherapy to improve the body’s overall resistance, suppressed by the disease and a course of chemotherapy.

Causes

Cancer has no exact cause. But at the same time, malignant lymphoma often forms against a background of weakened immunity; the main factors for its possible occurrence include:

• presence of HIV infection;
• exposure to radiation;
• genetic predisposition;
• systematic interaction with carcinogens, especially chemicals and heavy metals;
• Infectious mononucleosis;
• organ transplants and blood transfusions;
• age after 60 years.

Burkitt's lymphoma in most cases develops as a result of infection with the Epstein-Barr virus and this is a proven fact. Apart from this case, the rest of the list is only possible factors that may increase the risk of provoking the disease under certain conditions, but are not considered proven causes.

Clinical picture

Lymphoma has symptoms that usually appear when the brain tissue is compressed. These include:

• headaches and dizziness;
• visual, auditory and olfactory hallucinations;
• mental disorders;
• incoordination or loss of sensation in one part of the body or another;
• convulsions and sudden epileptic seizures;
• auditory and visual impairments;
• swelling of regional lymph nodes;
• sudden hemorrhages;
• vomit.

Along with these signs, when the cancer is not at the initial stage, general symptoms characteristic of oncology of any localization appear:

• loss of appetite;
• weakness;
• disability;
• sudden weight loss;
• temperature increase;
• fever.

In some cases there are no such manifestations, so you should be attentive to your body. Focal signs occur in 73% of patients, personal and mental changes in almost 50% of patients. A third of patients experience vomiting and headaches. Epileptic seizures occur in 15% of patients, and visual impairment occurs in only a few percent. Some patients report no or minor symptoms in the early stages of cancer. In the case when malignant lymphoma develops secondarily, against the background of metastasis from another organ, specific signs of the primary tumor may be inherent. In some cases, metastases are detected faster than the main tumor. Sometimes in the first stages, cancer does not show any signs.

Diagnostic measures

Non-Hodgkin's lymphoma is best diagnosed by magnetic resonance imaging. To make the method more informative, the patient is injected with a contrast agent, which detects cancer cells and accumulates in them, which allows you to get a more detailed picture of what is happening.

If cancer of any localization is suspected, a biopsy is a mandatory research method, allowing to obtain damaged tissue for histological examination. Further analysis determines the type and structure of the tumor, as well as its degree of malignancy. In addition to taking tissue samples from the tumor itself, a puncture is also made from spinal cord cells.

A chest x-ray is performed to view the condition of the lymphatic system of the mediastinum and thymus gland.

In addition to instrumental studies, laboratory blood tests are systematically required to monitor the body’s response to the tumor.

Therapy

Non-Hodgkin's lymphoma in the brain is difficult to treat because the organ has a blood-brain barrier that protects it from damage. Because of this, most methods are simply not able to radically influence the tumor.

Burkitt's lymphoma and its other varieties are better
Most can be treated with a course of chemotherapy. A course of mono or polychemotherapy may be prescribed. Depending on the type of lymphoma and its sensitivity to a particular group of drugs. Medicines are administered, as a rule, through a spinal puncture, since it is this approach that makes it possible to achieve the maximum effect from the drugs. Methotrexate is most often prescribed for a mono course of chemotherapy. If combined use of drugs is necessary, preference is given to Cytarabine, Temozolomide or Etoposide. The choice of medications remains only with the attending physician. A course of chemotherapy has many side effects and can often cause a deterioration in the patient’s well-being, but the risk over tumor growth justifies the advisability of its use. Side effects occur as a result of damage to healthy cells, since, unfortunately, chemotherapy cannot kill only cancer cells without affecting healthy ones. The frequency and intensity of certain manifestations depends on the dose and type of drug selected. Common side effects of chemotherapy include:

• nausea and vomiting;
• general weakness, malaise caused by anemia;
• hair loss;
• suppression of the body’s immune functions, resulting in greater susceptibility to infections;
• dry mouth and the formation of ulcers and wounds in its cavity;
• disorder in the gastrointestinal tract.

Radiation therapy

Radiation irradiation as an independent method is rarely used; more often it is combined with a course of chemotherapy or surgery. At a late stage of the disease, radiation therapy is used as a palliative method to alleviate the patient's condition by reducing the tumor and squeezing its healthy tissue. Side effects from radiation therapy can vary depending on the location of the radiation. Regarding the effects of radiation on the brain, negative manifestations may occur after several years. They are expressed, as a rule, in the form of neurological disorders. With the combined effects of a course of chemotherapy and radiation exposure, the negative consequences of the former may worsen.

Burkitt's lymphoma is practically incurable with surgery. The reason for this is the specific location and type of growth of the formation. Follicular lymphoma and many of its other types involve different brain cells, for example, the location of the main tumor may be in the cerebellum, and microscopic atypical cells may be scattered throughout the organ. In this regard, a radical type of operation is almost impossible to perform. They resort to surgery to remove the maximum part of the tumor in order to reduce tumor growth and take the material for a biopsy, and then use a course of chemotherapy or radiation to destroy the remaining atypical cells.

If the brain cancer is at an early stage and the size of the tumor is not impressive, and its location allows for surgery, we can talk about the success of the method and a positive outcome after surgery. But in any case, to make sure that all cancer cells are destroyed, despite the initial stage of the disease, the patient is recommended to undergo a course of chemotherapy to consolidate the result.

The prognosis depends on the stage, type of lymphoma and location. Young patients tolerate cancer a little easier and have better survival rates than older patients. Without urgent treatment, cancer localized in the mediastinum or brain very quickly affects their functioning and leads to death within a few months. With correctly selected, timely therapy, 40% of patients can overcome the five-year mark.

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