Modern classification of brain tumors. Common classification systems for brain tumors

1. tumors of neuroepithelial tissue(astrocytoma, glioblastoma, oligodendroglioma, epindymoma, etc.),

2. cranial nerve tumors(neurolemmoma, or neuroma of the vestibulocochlear nerve, etc.),

3. meningeal tumors(meningioma, etc.),

4. lymphomas and tumors of hematopoietic tissue, germ cell tumors(teratoma, etc.),

5. cysts and tumor-like processes(craniopharyngioma, etc.),

6. tumors of the sella region(pituitary adenoma, etc.),

7. tumor growth from nearby tissues, metastatic tumors, unclassified tumors.

In relation to the brain substance, tumors can be intracerebral(glioblastoma, etc.) and extracerebral(meningioma, etc.), by location to the tentorial tentorium of the cerebellum - supratentorial(tumors of the cerebral hemispheres, etc.) and subtentorial e (tumors of the cerebellum and brain stem).

Brain Tumor Clinic.

1. Headache

2. Vomiting

3. Visual impairment– occurs often when pituitary adenomas. 4. Dysfunction of cranial nerves– impaired sense of smell, impaired movements of the eyeballs, pain and/or numbness on the face, paresis of the facial muscles, hearing loss, imbalance, swallowing disorders, taste disorders, etc. 5. Focal symptoms

Diagnosis of brain tumors.

1. Careful neurological examination, including a detailed ophthalmological examination of acuity, visual fields and fundus. 2. CT (computed tomography), MRI (magnetic resonance imaging), angiography, etc., and also radioisotope methods 3. Electroencephalography(EEG) 4. Radiography

6. Ultrasonography used in children with open fontanelles.

7. Lumbar puncture.

Classification of spinal cord tumors.

I. Intramedullary tumors of the spinal cord –5%

1. Astrocytoma 40% - more common in childhood. 2. Ependymoma 37%. 3. Miscellaneous 30%

II. Intradural extramedullary tumors of the spinal cord(40%) 1. Meningiomas. 2. Neurofibromas. 3. Lipomas (are extramedullary, but with intramedullary extension). 4. Various (approximately 4% of spinal metastases).

III. Extradural spinal cord tumors arise in the vertebral bodies or epidural tissues(55%) 1. Metastatic (lung, breast, prostate cancer). 2. Primary tumors of the spine (very rare). 3. Chloroma: focal infiltration of leukemic cells. 4. Angiolipoma.

Clinical signs of spinal cord tumors vary greatly.

Pain is the most common symptom of intramedullary tumors of the spinal cord in adults and in 60-70% of patients pain is the first sign of the disease. Sensory or movement disorders are the first symptoms in 1/3 of cases.

Examination methods: 1. Examination by a specialist. 2. MRI. 3. CT and/or x-ray myelography. 4. Electromyography.

Treatment. Treatment of brain tumors is predominantly surgical and is often combined with radiation and chemotherapy. Many extracerebral tumors (meningiomas, neuromas, pituitary adenomas) can be completely removed. In most cases, this cannot be done with intracerebral tumors, so partial removal is performed, reducing compression of the brain substance, and then radiation and/or chemotherapy are used.

The basis of treatment for inoperable and metastatic tumors is radiation therapy and antitumor drugs. During the period of preoperative preparation and in the presence of cerebral edema, dehydration therapy is carried out - dexamethasone 4-6 mg intravenously 4 times a day or a 20% mannitol solution at the rate of 1 g/kg for a faster effect. For intense pain, non-narcotic analgesics are used in combination with dexamethasone. The prognosis depends on the histological structure and location of the tumor. The most effective surgical treatment of meningiomas and neuromas. For poorly differentiated tumors (glioblastomas, etc.) and brain metastases, the prognosis is poor. With a single metastasis to the brain, surgical treatment increases the patient's life expectancy.

82. 1. EEG is a method of recording the electrical activity of the brain through intact scalp, allowing one to judge its physiological maturity, functional state, the presence of focal lesions, general cerebral disorders and their nature. An electroencephalogram is a recording of the total electrical activity of the cells of the cerebral hemispheres.

EEG data are indicative in the diagnosis of epilepsy. With increased readiness for seizures, sharp waves and “peaks” appear on the EEG, which arise against the background of dysrhythmia and can be accompanied by hypersynchronization of the basic rhythm. In epilepsy, a major seizure causes an acceleration of EEG rhythms, a psychomotor seizure causes a slowdown in electrical activity, and a minor seizure ( absence seizure) - alternation of fast and slow oscillations (peak-wave complexes with a frequency of 3 per second).

During the interictal period, paroxysmal activity can be recorded on the EEG of patients with epilepsy, regardless of the type of seizure: more often - 3-4 oscillations/s. At tumors of the cerebral hemispheres(temporal, occipital, parietal localization) in 70-80% of cases, the EEG shows interhemispheric asymmetry with the presence of a focus of pathological activity in the form of polymorphic delta waves corresponding to the affected area.

At traumatic brain injury mild degree, short-term inhibition of alpha activity and the presence of delta waves are noted. These changes pass quickly. In severe traumatic brain injury, theta and delta waves dominate. Against this background, high-amplitude slow waves may appear in the form of flashes.

Many EEG changes may be nonspecific, i.e. their accurate interpretation is possible only taking into account the clinical picture of the disease and sometimes after additional examination. The results of the EEG depend on the age of the patient, the medications he is taking, the time of the last attack, the presence of tremor (shaking) of the head and limbs, visual impairment, and skull defects. All of these factors may influence the correct interpretation and use of EEG data.

2. MRI is the youngest radiologist. methods can create cross-sectional images of any part of the body. X-ray. emission-I no. Basic Accessories: strong magnet, radio transmitter, radio frequency receiver, tomograph. Exposure to a strong and uniform magnetic field changes the spins of the protons; they align in the direction of the field.

Advantages: non-invasive, absent. beam. load, three-dimensional x-ter is obtained. no image, natural contrast from moving blood. artifacts from bone tissue, high. diff soft fabrics.

Disadvantage: means. continue research (20-30 min), artifacts from rest. movement, violation heart rhythm in the presence of a pacemaker, unreliable. detection of stones, calcifications, high cost of equipment and its operation, specialist. requirements for premises (shielding from interference, separate power supply).

ABS. Opposite – cardiac pacemakers, clips on cerebral vessels, ferroimplants. ear. Relates. claustrophobia, weight more than 100 kg, presence of foreign objects. metal. items, take.

3. CT – layer-by-layer radiologist. research carried out on a computer. reconstruction of the image obtained by circular scanning of an object with a narrow beam of rays. radiation Tomographs: stepper, spiral, multispiral (64-slice). Advantages: absence of superposition (overlay of other organs), transverse layer orientation, high contrast resolution, determination of absorption coefficient, various types of image processing. Opposite: extremely heavy. comp. guys, let's take it.

4. Ultrasound Dopplerography (USDG) of the vessels of the neck and brain- a method for studying linear blood flow velocity (LBF), based on the Doppler effect (analysis of changes in the frequencies of returning ultrasound signals in comparison with those initially sent).

The method allows you to evaluate the linear blood flow velocity (LBV) in cm/sec in the carotid and vertebral arteries

There are practically no contraindications to the use of ultrasound scanning of the vessels of the neck and brain, with the exception of extensive soft tissue wounds in the study area, which prevents the application of the sensor.

5. Echoencephalography (EchoEG)- a method of non-invasive instrumental diagnostics, based on the reflection of ultrasound from the border of intracranial formations and environments with different acoustic densities (soft integument of the head, skull bones, meninges, medulla, liquor, blood). Pathological formations (foci of crush injury, foreign bodies, abscesses, cysts, hematomas, etc.) can also be reflective structures.

The most important indicator in echoencephalography (EchoEG) is the position of the midline structures of the brain (M – echo). Normally possible differences in the volume of the cerebral hemispheres allow for a physiological shift of M – echo up to 2 mm.

6. Rheoencephalography (REG) is a non-invasive method that studies volumetric fluctuations in the blood supply of the vessels of the brain and neck based on graphical recording of pulse-synchronous changes in resistance between electrodes applied to the scalp.

Using rheoencephalography (REG), one can judge the tone and elasticity of the blood vessels of the brain and neck, blood viscosity, the speed of pulse wave propagation, the speed of blood flow, evaluate latent periods, the duration and severity of regional vascular reactions.

7. Electromyography (EMG) and electroneurography (ENG)- these are valuable examination methods for studying the condition of peripheral nerves and muscles of the upper and lower extremities, neck, face, etc. EM method for studying bioelectric potentials arising in skeletal muscles humans and animals when excitation of muscle fibers; recording electrical activity of muscles. Electroneurography- registration of responses of peripheral nerves (VP ​​nerves) to their stimulation. To study the conduction velocity along the sensory nerve, one stimulating electrode and one recording electrode are used. 2]

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Tumors of the central nervous system are very diverse.

They are classified primarily by location, histological type and grade of malignancy.

Based on localization, tumors are located outside or inside the dura mater, inside (intracerebrally) or outside (extracerebrally) the medulla.

The latter include tumors of the meninges (meningiomas), cranial nerve roots (neurinomas), craniopharyngioma; most tumors growing into the cranial cavity from its bones and accessory cavities. Tumors can be located above the cerebellar tentorium (supratentorial) and below it (subtentorial).

Primary and secondary tumors are distinguished according to the place of origin (metastases from other organs and tumors growing into the cranial cavity), as well as according to localization in the lobes of the brain.
Classification brain tumors (BTM) According to the histological type and degree of malignancy during the development of neuro-oncology, it has changed several times and differed somewhat in different countries.

The most common classifications were Bailey and Cushing (1926, USA), L. I. Smirnov (1962, USSR), B. S. Khominsky (1969, USSR), Zülch (1956, 1980, Germany), Russi and Oberling (1948, France), etc.

Recently, the WHO classification (WHO) second revision (1993) has been generally accepted. In 2000, a new edition of this classification appeared, slightly different from the previous one, and in Russia - the classification of D. B. Matsko and A. G. Korshunov (1998).

Below we present the main variants of OGM that are of greatest importance in the clinic and meet the objectives of this publication.

Simplifying existing classifications as much as possible for practical purposes, GGMs can be grouped as follows:

I. Meningiomas.

II. Neuroectodermal tumors.

They constitute the most numerous group of brain tumors (up to 62%), among which the following variants are distinguished:

A) astrocytomas with many variants, including glioblastoma;
b) oligodendroglioma;
c) ependymoma;
d) choroid plexus papilloma;
e) neuronal tumors;
f) medulloblastoma;
g) tumors of the pineal gland.

III. Neuroma (neurilemmoma, schwannoma).

It should be noted that there is no general point of view about the unity of origin of these tumors. Thus, L. I. Smirnov, B. S. Khominsky, D. E. Matsko and A. G. Korshunov and others classify astrocytomas, glioblastomas, oligodendrogliomas and ependymomas as gliomas, but do not include choroid plexus tumors, but neuromas, or schwannomas are classified as peripheral “gliomas.” Our task is not to challenge this or that histological classification, but to present its most convenient and abbreviated form for clinicians.

IV. Tumors of the pituitary gland and remnants of the pituitary tract.

Among tumors of the adenohypophysis, according to tinctorial histological properties, chromophobe, eosinophilic and basophilic pituitary adenomas are distinguished.

Clinically, biochemically and immunohistochemically, adenomas are classified according to endocrine disorders:

1) without hormonal disorders;
2) prolactotropic;
h) adrenocorticotropic;
4) gonadotropic;
5) thyroid-stimulating;
6) polyhormonal, etc.

Craniopharyngiomas are considered separately.

V. Tumors of unknown origin.

VI. Cysts:

A) epidermoid cyst (cholesteatoma);
b) dermoid cyst;
c) colloid cyst of the third ventricle;
d) enterogenous cyst.

VII. Tumors growing into the cranial cavity:

A) chordoma;
b) chondroma;
c) chondrosarcoma, etc.

Conventionally (as space-occupying formations), the following two groups of neoplasms belong to tumors of the central nervous system.

VIII. Infectious granulomas.

IX. Vascular malformations.

For the convenience of students and young neurosurgeons, in the latest manual on neurosurgery by R. G. Grossmane and S. M. Loftus, OGMs are grouped as follows:

1. Tumors of the skull bones, membranes and cranial nerves:

A) tumors of the skull bones, 14 benign and 11 malignant variants;
b) meningeal tumors - meningiomas; by localization - 12, by histological structure - 13;
c) tumors of the cranial nerves, these include mainly acoustic neuroma, or schwannoma, which occurs in one case per 100,000 population. Tumors of other cranial nerves are rare.

2. Primary OGM: astrocytomas of various histostructures, glioblastoma, oligodendroglioma, ependymoma, subependymoma, choroid plexus tumors, ganglioma, dysplastic gangliocytoma (Lhermitte-Duclos disease), central neurocytoma, medulloblastoma, primary tumors of the pineal gland, primary lymph Oma and a number of others.

3. Cancer metastases to the brain.

According to S. S. Boring, in the United States every year there are 17,500 new cases of primary brain tumors and from 80,000 to 100,000 brain metastases. They are localized both in the cerebral hemispheres and in the cerebellum. They can be single or multiple, and sometimes there is contamination of the meninges (carcinomatosis).

They are found in patients known to have a cancerous tumor, but they can also be the first manifestation of a small and undiagnosed tumor of the bronchus, kidneys, etc. Let us consider the structure of the most important OGM.

WHO classification of brain tumors

The modern histological classification of tumors of the nervous system, developed by WHO experts in 1993, differs positively from the previous edition of 1979, primarily in that it quite fully reflects changes in views on the histogenesis and degree of malignancy of a number of neoplasms that occurred as a result widespread use in neuromorphology of a number of new techniques, in particular immunohistochemistry and molecular genetic analysis.

As a result, new histological forms of tumors were included in the latest edition of the classification - pleoform xanthoastrocytoma, dysembryoplastic neuroepithelial tumor, semitartal neurocytoma, etc., while malformed cell (monstrocellular) sarcoma was removed from the classification.

An important achievement of this version of the classification was also a clearer definition of several degrees of malignancy of “ordinary” astrocytic gliomas with a listing of their specific histological features, as well as the separation of pilocytic astrocytomas into a separate category. The assessment of glioblastomas as tumors of astroglial origin is absolutely fair and reasonable (in the 1979 WHO classification, these neoplasms were classified in the section “embryonic tumors” together with medulloblastomas).

The classification of meningeal neoplasms has undergone significant changes, among which two are of greatest importance:

1) biologically and clinically justified identification among meningiomas of an additional gradation of malignancy - atypical meningioma, which occupies an intermediate position between benign and malignant meningiomas;

2) removal from the classification of “hemangiopericytic” and “hemangioblastic meningiomas”; the former are classified as mesenchymal nonmeningothelial meningeal tumors, and the latter as hemangioblastomas.

We present the text of the WHO classification of the 2nd edition translated by D. E. Matsko and A. G. Korshunov.

Histological classification of tumors of the central nervous system (Kleihues P., Burger R. S., Scheithauer W. V., WHO, 1993)

1. Tumors from neuroepithelial tissue

1.1. Astrocytic tumors

1.1.1. Astrocytoma:

1.1.1.1. fibrillar
1.1.1.2. protoplasmic
1.1.1.3. gemistocytic (large cell)

1.1.2. Anaplastic (malignant) astrocytoma

1.1.3. Glioblastoma:

1.1.3.1. giant cell glioblastoma
1.1.3.2. gliosarcoma

1.1.4. Pilocytic astrocytoma
1.1.5. Pleomorphic xanthoastrocytoma
1.1.6. Subependymal giant cell astrocytoma (tuberous sclerosis)

1.2. Oligodendroglial tumors

1.2.1. Oligodendroglioma
1.2.2. Anaplastic (malignant) oligodendroglioma

1.3. Ependymal tumors

1.3.1. Ependymoma:

1.3.1.1. cellular
1.3.1.2. papillary
1.3.1.3. clear cell

1.3.2. Anaplastic (malignant) ependymoma
1.3.2. Myxopapillary ependymoma
1.3.3. Subependymoma

1.4. Mixed gliomas

1.4.1. Oligoastrocytoma
1.4.2. Anaplastic (malignant) oligoastrocytoma
1.4.3. Other

1.5. Tumors of the choroid plexus

1.5.1. Choroid plexus papilloma
1.5.2. Choroid plexus cancer

1.6. Neuroepithelial tumors of unknown origin

1.6.1. Astroblastoma
1.6.2. Polar spongioblastoma
1.6.3. Gliomatosis brain

1.7. Neuronal and mixed neuronal-glial tumors

1.7.1. Gangliocytoma
1.7.2. Dysplastic gangliocytoma of the cerebellum (Lermitte-Duclos)
1.7.3. Desmoplastic ganglioma in children (infantile)
1.7.4. Dysembryoplastic neuroepithelial tumor
1.7.5. Ganglioma
1.7.6. Anaplastic (malignant) ganglioma
1.7.7. Central neurocytoma
1.7.8. Paraganglioma of filum terminale

1.7.9. Olfactory neuroblastoma (esthesioneuroblastoma):

1.7.9.1. olfactory neuroepithelioma

1.8. Parenchymal tumors of the pineal gland

1.8.1. Pineocytoma
1.8.2. Pineoblastoma
1.8.3. Mixed/transitional tumors of the pineal gland

1.9. Embryonic tumors

1.9.1. Medulloepithelioma

1.9.2. Neuroblastoma:

1.9.2.1. ganglioneuroblastoma

1.9.3. Ependymoblastoma
1.9.4. Primitive neuroectodermal tumors

1.9.4.1. Medulloblastoma:

1.9.4.1.1. desmoplastic medulloblastoma
1.9.4.1.2. medullomyoblastoma
1.9.4.1.3. melanin-containing medulloblastoma

2. Tumors of the cranial and spinal nerves

2.1. Schwannoma (neurilemmoma, neuroma):

2.1.1. cellular
2.2.2. plexiform
2.2.3. melanin-containing

2.2. Neurofibroma (neurofibroma)

2.2.1. Limited (solitary)
2.2.2. Plexiform (mesh)

2.3. Malignant tumor of the peripheral nerve trunk (neurogenic sarcoma, anaplastic neurofibroma, “malignant schwannoma”):

2.3.1. epithelioid
2.3.2. malignant tumor of the peripheral nerve trunk with divergence of mesenchymal and/or epithelial differentiation
2.3.3. melanin-containing

3. Tumors of the meninges

3.1. Meningothelial cell tumors

3.1.1. Meningioma:

3.1.1.1. meningothelial
3.1.1.2. fibrous (fibroblastic)
3.1.1.3. transitional (mixed)
3.1.1.4. psammomatous
3.1.1.5. angiomatous
3.1.1.6. microcystic
3.1.1.7. secretory
3.1.1.8. clear cell
3.1.1.9. chordoid
3.1.1.10. rich in lymphoplasmacytic cells
3.1.1.11. metaplastic

3.1.2. Atypical meningioma
3.1.3. Papillary meningioma
3.1.4. Anaplastic (malignant) meningioma

3.2. Mesenchymal nonmeningothelial tumors

Benign tumors:

3.2.1. Osteochondral tumors
3.2.2. Lipoma
3.2.3. Fibrous histiocytoma
3.2.4. Other

Malignant tumors:

3.2.5. Hemangiopericytoma

3.2.6. Chondrosarcoma:

3.2.6.1. mesenchymal chondrosarcoma

3.2.7. Malignant fibrous histiocytoma
3.2.8. Rhabdomyosarcoma
3.2.9. Meningeal sarcomatosis
3.2.10. Other

3.3. Primary melanocytic lesions

3.3.1. Diffuse melanosis
3.3.2. Melanoma

3.3.3. Malignant melanoma:

3.3.3.1. Option: meningeal melanomatosis

3.4. Tumors of unknown histogenesis

3.4.1. Hemangioblastoma (capillary hemangioblastoma)

4. Lymphomas and tumors of hematopoietic tissue

4.1. Malignant lymphomas
4.2. Plasmacytoma
4.3. Granular cell sarcoma
4.4. Other

5. Tumors of germ cells (germ cell)

5.1. Germinoma
5.2. Embryonic cancer
5.3. Yolk sac tumor (endodermal sinus tumor)
5.4. Chorionic carcinoma

5.5. Teratoma:

5.5.1. immature
5.5.2. mature
5.5.3. with malignancy

5.6. Mixed germ cell tumors

6. Cysts and tumor-like lesions

6.1. Rathke's pouch cyst
6.2. Epidermal cyst
6.3. Dermoid cyst
6.4. Colloid cyst of the third ventricle
6.5. Enterogenous cyst
6.6. Neuroglial cyst
6.7. Granular cell tumor (chloristoma, pituicytoma)
6.8. Neuronal hamartoma of the hypothalamus
6.9. Nasal glial heterotopia
6.10. Plasmacytic granuloma

7. Tumors of the sella region

7.1. pituitary adenoma
7.2. pituitary cancer

7.3. Craniopharyngioma:

7.3.1. adamantinoma-like
7.3.2. papillary

8. Tumors growing into the cranial cavity

8.1. Paraganglioma (chemodectoma)
8.2. Chordoma
8.3. Chondroma

Brain tumors account for 10% of all neoplasms and 4.2% of all diseases of the nervous system. Spinal cord tumors are 6 times less common than brain tumors.

Etiology. Among the causes of the development of brain tumors is dysembryogenesis. It plays a role in the development of vascular tumors, malformations, and ganglioneuromas. The genetic factor plays a role in the development of vascular tumors and neurofibromas. The etiology of gliomas remains poorly understood. The development of neuroma of the vestibular-auditory nerve is associated with viral damage.

Classification of brain tumors

1. Biological: benign and malignant.

2. Pathogenetic: primary tumors, secondary (metastatic) from the lungs, stomach, uterus, breast.

3. In relation to the brain: intracerebral (nodular or infiltrative) and extracerebral with expansive growth.

4. Working neurosurgical classification: supratentorial, subtentorial, tuberogypophyseal.

5. Pathomorphological classification:

1. Neuroepithelial tumors (astrocytomas, oligodendrogliomas, ependymal and choroid plexus tumors, pineal gland tumors, neuronal tumors, medulloblastomas).

2. Tumors from nerve sheaths (acoustic neuroma).

3. Tumors of the meninges and related tissues (meningiomas, meningeal sarcomas, xanthomatous tumors, primary melanomas).

4. Blood vessel tumors (capillary hemangioblastoma)

5. Germ cell tumors (germinomas, embryonal cancer, chorionic carcinoma, teratoma).

6. Dysontogenetic tumors (craniopharyngioma, Rathke's pouch cyst, epidermoid cyst).

7. Vascular malformations (arteriovenous malformation, cavernous angioma).

8. Tumors of the anterior lobe of the pituitary gland (acidophilic, basophilic, chromophobic, mixed).

9. Adenocarcinomas.

10. Metastatic (6% of all brain tumors).

Glioma is a specific tumor of the nervous system, consisting of brain matter. Gliomas occur in adults and the elderly. The degree of malignancy of gliomas depends on the type of glioma cells. The less differentiated the tumor cells, the more malignant the course is observed. Gliomas include glioblastomas, astrocytomas and medulloblastomas.

Glioblastoma has infiltrating growth. This is a malignant tumor. Glioblastomas range in size from a nut to a large apple. Most often, glioblastomas are single, much less often - multiple. Sometimes cavities form in gliomatous nodes, sometimes calcium salts are deposited. Sometimes hemorrhage occurs inside the glioma, then the symptoms resemble a stroke. The average life expectancy after the first signs of the disease appear is about 12 months. With radical removal, tumor recurrences often occur.

Astrocytoma. They have benign growth. Growth continues slowly and for a long time. Large cysts form inside the tumor. Average life expectancy is about 6 years. Once the tumor is removed, the prognosis is favorable.

Medulloblastoma. A tumor consisting of undifferentiated cells that have no signs of either neurons or glial elements. These tumors are the most malignant. They are found almost exclusively in the cerebellum in children (usually boys) aged about 10 years.

Other gliomas include oligodendroglioma. This is a rare, slow-growing tumor. Has relatively benign growth. Found in the cerebral hemispheres. May be subject to calcification. Ependymoma develops from ventricular ependyma. It is located in the cavity of the fourth ventricle or, less commonly, in the lateral ventricle. Has benign growth.

Meningiomas make up 12-13% of all brain tumors and occupy the second place in frequency after gliomas. They develop from the cells of the arachnoid membrane. They have benign growth. They are located outside the brain tissue along the venous sinuses. They cause changes in the underlying bones of the skull: the formation of usuria, endostosis occurs, and the diploetic veins expand. Meningiomas are more common in women aged 30-55 years. Meningiomas are divided into convexital and basal. In some cases, meningiomas calcify and develop into psammomas.

Tumors of the pituitary region make up 7-18% of all brain tumors. The most common are craniopharyngiomas and pituitary adenomas.

Craniopharyngioma develops from embryonic remains of gill arches. Tumor growth is expansive. Located in the area of ​​the sella turcica. Forms cystic cavities. Occurs in the first two decades of life.

Pituitary adenomas develop from the glandular pituitary gland, i.e. front They develop in the cavity of the sella turcica. There are basophilic, eosinophilic and chromophobic depending on the cell type. When malignant, the tumor is called adenocarcinoma. As the tumor grows, it destroys the back of the sella turcica, the diaphragm and grows into the cranial cavity. May put pressure on the chiasm, hypothalamus and cause related symptoms.

Metastatic formations account for 6% of all brain tumors. Sources of metastasis are bronchogenic lung cancer, breast, stomach, kidney, and thyroid cancer. The routes of metastasis are hematogenous, lymphogenous and cerebrospinal fluid. Most often, metastases are single, less often multiple. They are located in the brain parenchyma, less often in the bones of the skull.

Brain Tumor Clinic

The clinical picture of brain tumors consists of three groups of symptoms. These are general cerebral symptoms, focal and distant symptoms.

General cerebral symptoms occur due to increased intracranial pressure. A complex of cerebral symptoms forms the so-called hypertension syndrome. Hypertension syndrome includes headache, vomiting, papilledema, changes in vision, mental disorders, epileptic seizures, dizziness, changes in pulse and respiration, and changes in the cerebrospinal fluid.

Headache - one of the most common symptoms of a brain tumor. It occurs as a result of increased intracranial pressure, impaired blood and liquor circulation. At the beginning, headaches are usually local, caused by irritation of the dura mater, intracerebral and meningeal vessels, as well as changes in the bones of the skull. Local pain can be boring, pulsating, jerking, or paroxysmal in nature. Identifying them is of some importance for topical diagnosis. With percussion and palpation of the skull and face, pain is noted, especially in cases of superficial tumor location. Expanding headaches often occur at night and early in the morning. The patient wakes up with a headache that lasts from several minutes to several hours and appears again the next day. Gradually, the headache becomes prolonged, diffuse, spreads throughout the head and can become permanent. It can intensify with physical stress, anxiety, coughing, sneezing, vomiting, bending the head forward and defecating, depending on the posture and position of the body.

Vomit appears when intracranial pressure increases. With tumors of the fourth ventricle, medulla oblongata, and cerebellar vermis, vomiting is an early and focal symptom. It is characterized by its occurrence at the height of a headache attack, the ease of occurrence, more often in the morning, when changing the position of the head, there is no connection with food intake.

Congested optic discs arise due to increased intracranial pressure and the toxic effect of the tumor. The frequency of their appearance depends on the location of the tumor. They are almost always observed with tumors of the cerebellum, fourth ventricle and temporal lobe. They may be absent in tumors of the subcortical formations; they appear late in tumors of the anterior part of the brain. Transient blurred vision and a progressive decrease in its acuity indicate stagnation and possible incipient atrophy of the optic discs. In addition to secondary atrophy of the optic nerves, primary atrophy can also be observed when the tumor exerts direct pressure on the optic nerves, chiasm or initial segments of the optic tracts in cases of its localization in the area of ​​the sella turcica or at the base of the brain.

General cerebral symptoms of a tumor also include epileptic seizures, mental changes, dizziness, and slow pulse.

Epileptic seizures may be caused by intracranial hypertension and the direct effect of the tumor on the brain tissue. Seizures can appear in all stages of the disease (up to 30%), often serve as the first clinical manifestations of the tumor and precede other symptoms for a long time. Seizures occur more often with tumors of the cerebral hemispheres located in the cortex and close to it. Seizures are less common with deep-seated tumors of the cerebral hemispheres, brain stem and posterior cranial fossa. Seizures are observed more often at the onset of the disease, with the slow growth of a malignant tumor, than with its more rapid development.

Mental disorders most often occur in middle and old age, especially when the tumor is located in the anterior lobes of the brain and the corpus callosum. Patients are depressed, apathetic, drowsy, often yawn, get tired quickly, and are disoriented in time and space. There may be memory problems, mental slowness, difficulty concentrating, irritability, mood changes, agitation, or depression. The patient may be stunned, as if separated from the outside world - “loaded”, although he can answer questions correctly. As intracranial pressure increases, mental activity ceases.

Dizziness often occurs (50%) due to congestion in the labyrinth and irritation of the vestibular stem centers and temporal lobes of the cerebral hemispheres. Systemic vertigo with rotation of surrounding objects or one’s own displacement of the body is relatively rare, even with acoustic neuroma and tumor of the temporal lobe of the brain. Dizziness that occurs when the patient changes position may be a manifestation of ependymoma or metastasis to the fourth ventricle.

Pulse with brain tumors it is often labile, sometimes bradycardia is detected. Blood pressure may increase with a rapidly growing tumor. In a patient with a slowly growing tumor, especially of a subtentorial localization, it is often reduced.

Frequency and character breathing also changeable. Breathing can be rapid or slow, sometimes with a transition to the pathological type (Cheyne-Stokes, etc.) in the late stage of the disease.

Cerebrospinal fluid flows out under high pressure, transparent, often colorless, sometimes xanthochromic. Contains an increased amount of protein with normal cellular composition.

The greatest severity of hypertension syndrome is observed with subtentorial tumors, extracerebral localization with expansive growth.

Focal symptoms associated with the direct effect of the tumor on the adjacent area of ​​the brain. They depend on the location of the tumor, its size and stage of development.

Tumors of the anterior central gyrus. In the initial stages of the disease, Jacksonian-type seizures are observed. Convulsions begin in a certain part of the body, then spread according to the topical projection of body parts to the anterior central gyrus. Generalization of a convulsive seizure is possible. As they grow, convulsive phenomena begin to be accompanied by central paresis of the corresponding limb. When the lesion is localized in the paracentral lobule, lower spastic paraparesis develops.

Tumors of the posterior central gyrus. Irritation syndrome affects sensory Jacksonian epilepsy. There is a feeling of crawling in certain areas of the body or limbs. Paresthesia can spread to the entire half of the torso, or to the entire body. Symptoms of loss may then follow. Hypesthesia or anesthesia occurs in areas corresponding to the cortical lesion.

Frontal lobe tumors. They can be asymptomatic for a long time. The following symptoms are most characteristic of a frontal lobe tumor. Mental disorders. They are expressed by decreased initiative, passivity, lack of spontaneity, indifference, lethargy, decreased activity and attention. Patients underestimate their condition. Sometimes there is a tendency towards flat jokes (moria) or euphoria. Patients become untidy and urinate in inappropriate places. Epileptic seizures can begin with turning the head and eyes to the side. Frontal ataxia is detected on the side opposite to the lesion. The patient staggers from side to side. There may be loss of the ability to walk (abasia) or stand (astasia). Smell disorders are usually unilateral. Central paresis of the facial nerve occurs due to tumor pressure on the anterior central gyrus. This is most often observed with tumors localized in the posterior part of the frontal lobe. When the frontal lobe is damaged, the phenomenon of obsessive grasping of objects (Janiszewski's symptom) may occur. When the tumor is localized in the posterior part of the dominant hemisphere, motor aphasia occurs. In the fundus, changes can either be absent, or there may be bilateral congestive nipples of the optic nerves, or a congestive nipple on one side and atrophic on the other (Foerster-Kennedy syndrome).

Tumors of the parietal lobe. Hemiparesis and hemihypesthesia develop most often. Among sensory disorders, the sense of localization suffers. Astereognosis occurs. When the left angular gyrus is involved, alexia is observed, and when the supramarginal gyrus is affected, bilateral apraxia is observed. When the angular gyrus suffers at the junction with the occipital lobe of the brain, visual agnosia, agraphia, and acalculia develop. When the lower parts of the parietal lobe are damaged, a violation of right-left orientation, depersonalization and derealization appears. Objects begin to appear large or, conversely, smaller, and patients ignore their own limbs. When the right parietal lobe suffers, anosognosia (denial of one's illness) or autotopagnosia (disturbance in the body diagram) may occur.

Temporal lobe tumors. The most common aphasia is sensory, amnestic, and alexia and agraphia may occur. Epileptic seizures are accompanied by auditory, olfactory, and taste hallucinations. Visual disturbances in the form of quadrant hemianopia are possible. Sometimes there are attacks of systemic dizziness. Large tumors of the temporal lobe may cause herniation of the temporal lobe medulla into the notch of the tentorium cerebellum. This is manifested by oculomotor disorders, hemiparesis or parkinsonism. Memory disorders most often occur with damage to the temporal lobe. The patient forgets the names of relatives, loved ones, and the names of objects. General cerebral symptoms in tumors of the temporal lobes are significantly expressed.

Tumors of the occipital lobe. They are rare. The most common are visual disturbances. Optical agnosia develops.

Brain stem tumors. Causes alternating paralysis.

Tumors of the cerebellopontine angle. As a rule, these are acoustic neuromas. The first sign may be noise in the ear, then there is a decrease in hearing up to complete deafness (otiatric stage). Then signs of damage to other cranial nerves appear. These are V and VII pairs. Trigeminal neuralgia and peripheral paresis of the facial nerve occur (neurological stage). In the third stage, the posterior cranial fossa is blocked with pronounced hypertensive phenomena.

Pituitary tumors. They cause bitemporal hemianopsia due to compression of the chiasm. Primary atrophy of the optic nerves occurs. Endocrine symptoms, adipose-genital dystrophy, and polydipsia develop. On radiographs, the sella turcica is enlarged in size.

"Symptoms at a Distance" This is the third group of symptoms that can occur with brain tumors. They should be taken into account, as they can lead to errors in determining the location of the tumor. Most often this is due to unilateral or bilateral damage to the cranial nerves, especially the abducens, less often the oculomotor nerve, as well as pyramidal and cerebellar symptoms in the form of ataxia and nystagmus.

Diagnostics. It is carried out on the basis of the clinical picture of the disease. Additional methods include liquor diagnostics. Its value is now decreasing. The main diagnosis is carried out using CT and MRI.

Treatment

Dehydration therapy with glucocorticosteroids is carried out. By reducing the swelling of the underlying substance of the brain, some regression of symptoms may be observed. Osmodiuretics (mannitol) can be used as diuretics.

Surgical treatment is most effective for extracerebral tumors (meningiomas, neuromas). For gliomas, the effect of surgical treatment is lower and a neurological defect remains after surgery.

Types of surgical interventions:

 Craniotomy is performed on superficial and deep tumors.

 Stereotactic intervention is performed if the tumor is deep and produces minimal clinical manifestations.

 The tumor can be radically removed and part of it resected.

Other treatment methods include radiation therapy and chemotherapy.

In each case, an individual approach is taken.

Brain cancer is a variety of abnormal growths that occur due to abnormal growth, development and division of brain cells. The classification of brain tumors includes benign and malignant neoplasms; they are not divided according to general principles. This is explained by the fact that both types of brain tumor put equal pressure on its tissue, since as it grows the skull cannot move to the sides.

Benign and malignant brain tumors

III. Ependymomas

Ependermal cells lining the inside of the ventricles of the brain, as well as cells located in the space of the brain and spinal cord filled with a liquid substance, give rise to ependymomas. Ependymomas of grades 2 and 3 are considered malignant. They develop in any area of ​​the brain and spine and metastasize to the spinal cord through the cerebrospinal fluid.

Ependymomas are more common in women, of which 60% survive less than 5 years. Most often, the tumor is located in the posterior fossa of the brain (the back of the skull). At the same time, intracranial pressure increases, gait becomes clumsy and unstable. The patient has difficulty swallowing, speaking, writing, solving problems, and walking. Gait, behavior and personality changes. Patients become lethargic and irritable.

IV. Medulloblastomas

They develop from embryonic cells in the cranial fossa, most often in children. Tumors are pale brown in color and in some places have a clear demarcation from the brain tissue. Due to infiltrative growth, they are able to grow into surrounding tissues. Lead to hydrocephalus by blocking the fourth ventricle of the brain. Medulloblastomas (melanotic and muscle fiber medullomyoblastoma) often metastasize to the spinal cord.

Tumors of stages 2-4 are considered malignant.

The most common symptoms in patients are:

• headache syndromes;
• strange nausea and vomiting;
• problems walking, loss of balance;
• slow speech, impaired writing;
• drowsiness and lethargy;
• weight loss or gain.

V. Tumors of the pineal gland

The pineal gland is designed to perform endocrine functions. It consists of neuronal cells (pinocytes). They are associated with light-sensitive retinal cells. Tumors of the pineal gland are rare in children aged 13-20 years. These include:

• pineocytoma - a slow-growing tumor consisting of mature pinealocytes, located in the pineal gland;
• pineoblastoma – a tumor with a high degree of malignancy and the ability to metastasize;
• neoplasm of the pineal gland parenchyma with an unpredictable course. Happens more often in children.

Secondary cancers include metastatic neoplasms. Sometimes it is impossible to determine the source of metastases, so such tumors are called formations of unknown origin. Symptoms of secondary tumors are identical to those of primary cancer.

Classification according to the TNM system and stage of brain cancer

• T (tumor, tumor) - the stage at which the tumor reaches a certain size and size:

1. T1 – value is assigned to neoplasms that have dimensions: up to 3 cm for types of cancer of the subcerebellar zone; up to 5 cm – for supracerebellar formations;
2. T2 – when the unit exceeds the above dimensions;
3. T3 – tumor grows into the ventricles;
4. T4 – the tumor is large and spreads to the second half of the brain.

• N (nodes) - the stage at which the degree of involvement of the lymph nodes in tumor processes is determined;
• M (metastasis, metastasis) - stage of metastases.

As for the N and M indicators, they do not have much significance in this situation; it is important in this situation to know what the size of the tumor is, since the size of the skull is limited. The appearance of one or more formations leads to serious disruptions in brain function. There is a danger of compression and disruption of the functions of individual elements.

Over a period of time, the classification was expanded with two more characteristics:

• G (gradus, degree) - degree of malignancy;
• P (penetration, penetration) - the degree of germination of the wall of a hollow organ (used only for tumors of the gastrointestinal tract).

• Stage 1 indicates that the tumor is small in size and grows quite slowly. Under the microscope, almost normal cells appear. This type is quite rare and can be removed by surgery.
• Stage 2 – the tumor grows slowly. It differs from the first degree in the size of the tumor and the structure of the cells.
• Stage 3 is a tumor that is growing rapidly and spreading rapidly. The cells are significantly different from normal ones.
• Stage 4 is a fast-growing tumor that metastasizes throughout the body. It cannot be treated.

Informative video:

SCIENTIFIC REVIEWS

© BATOROEV Y.K. - 2009

ABOUT NEW NOSOLOGICAL FORMS OF THE WHO CLASSIFICATION OF TUMORS OF THE CENTRAL NERVOUS SYSTEM (fourth edition, 2007)

Yu.K. Batoroev

(Irkutsk State Institute for Advanced Training of Physicians, Rector - Doctor of Medical Sciences, Prof. V.V. Shprakh, department

oncology, head - Doctor of Medical Sciences, Prof. V.V. Dvornichenko)

Resume. An original translation of the WHO classification of tumors of the central nervous system, reissued in 2007, is presented, including a description of some new nosologies taking into account modern morphogenetic concepts. A gradation of the degree of malignancy and ICD-oncological codes is given. A brief description of hereditary tumor syndromes associated with the occurrence of tumors of the nervous system is given.

Key words: histological WHO classification, central nervous system tumors.

ABOUT NEW NOSOLOGICAL FORMS OF THE FOURTH EDITION WHO-CLASSIFICATION OF THE TUMORS OF THE CENTRAL NERVOUS SYSTEM (2007)

Y.K. Batoroev (Irkutsk State Institute for Medical Advanced Studies)

Summary. There has been presented the original translation of the World Health Organization (WHO) classification of tumors of the central nervous system, translation of the fourth edition published in 2007, lists several new nosological forms. Histological variants were added if there was evidence of a different age distribution, location, genetic profile or clinical behavior. The WHO grading scheme and the sections on genetic profiles were updated and predisposition syndrome was added to the list of familial tumor syndromes typically involving the nervous system.

Key words: WHO classification, tumors of the central nervous system.

In the work of oncological institutions, unified rubrication, nomenclature and classification are extremely important. This is the language of communication of oncologists, chemotherapists, radiologists, surgeons, internists of various profiles and pathomorphologists; it should be as simple, clear, accessible and international as possible. When starting to translate the WHO classification of tumors of the nervous system (NS), the authors clearly understood the current state of the pathological and statistical services in Russia - its capital, regional centers and in the outback. Due to the lack of modern WHO classifications in Russian, most pathologists and medical statisticians in our country use a variety of tumor classifications. Many pathohistologists use the outdated “Geneva” WHO classification of tumors of the central nervous system from 1979, the classification from the excellent monograph for those years (1969) by B.S. Khominsky, and medical statistics - ICD-10. As the facts, sometimes paradoxical, obtained using new molecular biological methods for diagnosing tumors accumulated and were comprehended, the need to revise the WHO classifications of tumors became obvious. In 1993, under the leadership of P. Kleuhues, P. Burger and B. Shceithauer, a revised, second version of the classification of CNS tumors appeared. Since 2000, the International Agency for Research on Cancer, Lyon, France (International Agency for Research Cancer - IARC), which is a structural unit of WHO, began publishing the third, and in 2007 - the fourth series of the so-called “blue books” , which got their name because of the characteristic logo) -

histological WHO classifications of tumors of various organs. The first edition contained 25 volumes, the third - 9, which covered tumors of almost all organs and tissues.

Classification of NS tumors, third and fourth editions, differs significantly from both the first (1979) and the second (1993). If the first edition, back in the Soviet Union, was translated into Russian and replicated by the publishing house "Medicine", then the second edition remained little known. The classification itself was translated by St. Petersburg pathohistologist from the Neurosurgical Institute D.N. Matsko, who accompanied this translation with a brief commentary in the 1996 anniversary collection dedicated to M.F. Glazunov. But it remained unknown to most domestic pathomorphologists, neurosurgeons and oncologists. Later, in 1998, D.N. Matsko, in collaboration with A.G. Korshunov, published “Atlas of Tumors of the Central Nervous System,” which was based on the author’s original classification, not much different from the WHO classification of 1993. It has not lost its relevance to this day, and can be considered an “adapted” version of the WHO classification to Russian conditions.

Due to the rapid development of molecular biological methods, especially immunomorphological ones, in the 1980-90s the histogenesis of many tumors was determined, which led not only to the identification of new nosological units, but also to the reclassification of some others. Thus, glioblastomas, after revealing their astroglial nature, were moved from the group of “embryonic” tumors to “astrocytic” tumors, thereby closing the logical chain of their oncological

genesis: astrocytoma ^ anaplastic astrocytoma ^ glioblastoma. Previously, it was erroneously believed that glioblastoma histogenetically can originate from both astroglia and oligodendroglia and even from ependyma. The group of meningiomas was significantly changed, which were divided into three groups according to the degree of malignancy (typical, atypical and anaplastic). The following were added to typical meningiomas: microcystic, secretory, metaplastic, lymphoplasmacytic. Clear cell and chordoid have been added to the atypical group, and papillary and rhabdoid to the anaplastic group. From the group of meningiomas in general, hemangioblastic and heman-hypercytic meningiomas were removed, which were converted into mesenchymal tumors of the membranes.

The third and fourth editions of WHO classification books of this series are generally fundamentally different from the previous two. These differences, firstly, relate to the number of participants. If earlier the circle of participants was limited to 20-25 people: 12 managers, 10-12 experts and the same number of reviewers, now the number of participants in each book (there are nine in total so far) ranges from 77 to 143. The work on each volume is supervised by such “ blue book" two or three editors, and the most titled co-authors (about twenty) are invited to take part in the inception and final meetings where the main decisions are made. Secondly, the format and volume of the publication have increased, and the remaining, as a whole, previous logo has been supplemented with the most characteristic color illustrations. On the cover of the books of the third edition, instead of the previous title “International Histological Classification of Tumors,” “Pathology and Genetics of Tumors” appears, which emphasizes fundamentally new approaches to clarifying the diagnosis of tumors. At the beginning of each volume, the classifications themselves are given, indicating the codes of the International Classification of Diseases - Oncology (ICD/O). A four-digit ICD/O code is assigned to each oncological unit, and the degree of its malignancy is indicated through an oblique line (0 - benign tumor, 1 - tumor of intermediate degree of malignancy, locally aggressive or rarely metastasizing, 2 - carcinoma “in situ”, 3 - malignant tumor). An entire chapter is devoted to a separate nosological unit, indicating its authors. At the beginning of each chapter, a definition of nosology is given, its previous names, synonyms, ICD/O code, then the frequency of occurrence, favorite localization, age and gender. The clinical symptoms that are characteristic of it, the features of X-ray, CT, and ultrasound images, the criteria for classification and staging are given in detail. After this, the appearance of the gross specimen of the removed tumor is described, a detailed histological picture is given, indicating some criteria, such as the mitotic index or the area of ​​necrosis, necessary to determine the degree of malignancy. The following describes the previous conditions, the immunohistochemical profile, provides data from cytogenetic and molecular genetic studies, as well as morphological criteria that determine recurrence, survival and prognosis. Descriptions are accompanied mainly by

richly colored illustrations. At the end of each book there is a list of referenced articles. This list includes from two to three thousand sources. The book ends with a list of authors in alphabetical order, which provides postal and email addresses indicating their place of work and position.

The volume we are discussing, “WHO Classification of CNS Tumors,” was published in 2007, edited by a group of authors led by American pathologist from Boston D. Louis. 74 experts from 20 countries took part in its creation, including from Russia - A.G. Korshunov, head Department of Pathomorphology, Neurosurgical Institute named after. N.N. Burdenko.

We present our translation of the classification, familiarity with which will be useful for pathomorphologists, neurosurgeons, neurologists, oncologists and medical statisticians (Table 1).

In this version, compared to previous classifications, there have been significant changes in the range of tumors discussed: 1) in addition to tumors of the central nervous system and tumors of the cranial nerves, tumors of the peripheral nervous system, which were previously discussed in the classification of soft tissue tumors, from where they were derived, are now also considered; 2) pituitary adenomas, which are considered tumors of the endocrine system, are also excluded; 3) the classification does not include, but considers in detail, hereditary tumor syndromes involving the central nervous system and chromosomal aberrations are indicated with mapping of key oncogenes and suppressor genes.

It should be noted that there is a double system for grading the degree of malignancy of CNS tumors. The first codes according to the ICD/O system, and this 4-digit code is shown in the table on the right, where the degree of malignancy is indicated by numbers separated by a fraction: /0 - benign tumor, /1 - tumor of intermediate degree of malignancy, /2 - carcinoma “in situ”, /3 - malignant tumor. In addition, it is necessary to evaluate the tumor on another scale - a gradation of the degree of malignancy, developed specifically for tumors of the central nervous system, the foundations of which were laid by the outstanding American neuropathohistologist J.W. Kernogen in 1949. Its development was due to the fact that the formal morphological gradation the degree of malignancy of tumors, for example, such as for epithelial carcinomas, proposed by Broders (A.C. Broders, 1948), for tumors of the central nervous system is not entirely acceptable for several reasons:

The unimpeded growth of a tumor, even a completely benign one, within the cranium can lead to compression of vital brain structures and lead to death, which, of course, indicates the clinical malignancy of the process;

This course of the process can be caused by any tumor, regardless of its histological structure and degree of malignancy;

A tumor of any histotype and any degree of malignancy, even very small sizes, can cause occlusive hydrocephalus with any, the most severe consequences;

When assessing the degree of malignancy of CNS tumors, some general morphological criteria for malignancy

Table 1

WHO classification of tumors of the central nervous system (2GG7)

Type of tumors Code Degree of malignancy

ICD/O quality Yu)

1. NEUROEPITHELAL TUMORS

1.1. Astrocytic tumors

Pilocytic astrocytoma 9421/1 G = I

Pilomyxoid astrocytoma 9425/3 G = II

Subependymal giant cell astrocytoma 9384/3 G = I

Pleomorphic xanthoastrocytoma 9424/3 G = I

Diffuse astrocytoma 9420/3 G = II

fibrillar 9420/3 G = II

protoplasmic 9410/3 G = II

mast cell 9411/3 G = II

Anaplastic astrocytoma 9401/3 G = III

Glioblastoma 9440/3 G = IV

Giant cell glioblastoma 9441/3 G = IV

Gliosarcoma 9442/3 G = IV

Gliomatosis brain 9381/3 G = III

1.2. Oligodendroglial tumors

Oligodendroglioma 9450/3 G = II

Anaplastic oligodendroglioma 9451/3 G = III

1.3. Oligoastrocytic tumors

Oligoastrocytoma 9382/3 G = II

Anaplastic oligoastrocytoma 9382/3 G = III

1.4. Ependymal tumors

Myxopapillary ependymoma 9394/1 G = I

Subependymoma 9381/1 G = I

Ependymoma 9391/3 G = II

cellular 9391/3 G = II

papillary 9391/3 G = II

clear cell 9391/3 G = II

tanicytic 9391/3 G = II

Anaplastic ependymoma 9392/3 G = III

1.5. Choroid plexus tumors

Choroid plexus papilloma 9390/0 G = I

Atypical papilloma of the choroid plexus 9390/1 G = II

Choroid plexus carcinoma 9390/3 G = III

1.6. Other neuroepithelial tumors

Astroblastoma 9430/3 unclear

Chordoid glioma of the third ventricle 9444/1 G = II

Angiocentric glioma 9431/1 G = I

1.7. Neuronal and mixed neuronal-glial tumors

Dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease) 9493/0 G = I

Infantile desmoplastic astrocytoma/ganglioglioma 9421/1 G = I

Dysembryoplastic neuroepithelial tumor 9413/0 G = I

Gangliocytoma 9492/0 G = I

Ganglioglioma 9505/1 G = I

Anaplastic ganglioglioma 9505/3 G = III

Central neurocytoma 9506/1 G = II

Extraventricular neurocytoma 9506/1 G = II

Cerebellar liponeurocytoma 9506/1 G = II

Papillary glioneuronal tumor 9509/1 G = I

Rosette-forming glioneuronal tumor of the fourth ventricle 9509/1 G = I

Spinal paraganglioma (terminal filum cauda equina) 8660/1 G = I

1.9. Pineal tumors

Pineocytoma 9361/1 G = I

Tumor of the pineal gland of intermediate degree of malignancy 9362/3 G = II-III

G = II-III Pineoblastoma 9362/3 G = IV

Papillary tumor of the pineal gland 9395/3 G = II-III

Tumor of the parenchyma of the pineal gland intermedia 9362/1 G = III

degree of malignancy

1.11. Embryonic tumors

Medulloblastoma 9470/3 G = IV

continuation of table. 1

Desmoplastic/nodular medulloblastoma Medulloblastoma with marked nodularity Anaplastic medulloblastoma Large cell medulloblastoma Melanotic medulloblastoma Primitive neuroectodermal tumor of the central nervous system (PNET) Neuroblastoma of the central nervous system Ganglioneuroblastoma of the central nervous system Medulloepithelioma Ependymoblastoma Atypical teratoid/rhabdoid tumor 9471/3 9471/3 9474/3 9474/3 9472/3 9473/3 9473/3 9490/3 9501/3 9392/3 9508/3 G = IV G = IV G = IV G = IV G = IV G = IV G = IV G = IV G = IV G = IV G = IV

2. TUMORS OF THE CRANIAL AND PARASPINAL NERVES

2.1. Schwannoma (neurilemmoma, neuroma) 9560/0 G = I

cellular 9560/0 G = І

plexiform 9560/0 G = І

melanotic 9560/0 G = І

2.2. Neurofibroma 9540/0 G = I

plexiform 9550/0 G = І

2.3. Perineuroma 9571/0 G = I

intraneural perineuroma 9571/0 G = І

malignant perineuroma 9571/0 G = I

2.4. Malignant peripheral nerve tumor (MPT) 9540/3 G=PI-GV

epithelioid 9540/3 G=IP-IV

with mesenchymal differentiation 9540/3 G=IP-IV

melanotic 9540/3 G=IP-IV

with glandular differentiation 9540/3 G=IP-IV

3. TUMORS OF THE MEMONS

3.1. Meningothelial cell tumors

Typical meningioma 9530/0 G =І

meningotheliomatous 9531/0 G =І

fibrous 9532/0 G =І

transitional 9537/0 G =І

psammomatous 9533/0 G =І

angiomatous 9534/0 G =І

microcystic 9530/0 G =І

secretory 9530/0 G =І

with an abundance of lymphocytes 9530/0/ G =І

metaplastic 9530/0 G =І

Atypical meningioma 9539/1 G = II

Chordoid meningioma 9538/1 G = II

Clear cell meningioma 9538/1 G = II

Anaplastic meningioma 9530/3 G = III

Rhabdoid meningioma 9538/3 G = III

Papillary 9538/3 G = III

3.2. Mesenchymal tumors of the membranes (non-meningotheliomatous)

Lipoma 8850/0 G =І

Angiolipoma 8861/0 G =І

Hibernoma 8880/0 G =І

Liposarcoma 8850/3 G = III

Solitary fibrous tumor 8815/0 G =І

Fibrosarcoma 8810/3 G = III

Malignant fibrous histiocytoma 8830/3 G = III

Leiomyoma 8890/0 G =І

Leiomyosarcoma 8890/3 G = III

Rhabdomyoma 8990/0 G =І

Rhabdomyosarcoma 8900/3 G = III

Chondroma 9220/0 G =І

Chondrosarcoma 9220/3 G = III

Osteoma 9180/0 G =І

Osteosarcoma 9180/3 G = III

Osteochondroma 0921/1 G =І

Hemangioma 9120/0 G =І

Epithelioid hemangioendothelioma 9133/1 G =II

Hemangiopericytoma 9150/1 G=II

end of table 1

Anaplastic hemangiopericytoma 9150/3 o=sh

Angiosarcoma 9120/3 o=sh

Kaposi's sarcoma 9140/3 o=sh

Ewing's sarcoma 9364/3 v=gu

3.3. Primary melanotic lesions

Diffuse melanocytosis 8728/0

Melanocytoma 8727/1

Malignant melanoma 8720/3

Meningeal melanomatosis 8728/3

3.4. Other tumors related to the membranes

Hemangioblastoma 9661/1

3.5. Lymphomas and tumors of the hematopoietic system

Malignant lymphoma 9590/3

Plasmacytoma 9731/3

Granulocytic sarcoma 9930/3

3.6. Germ cell tumors

Germinoma 9064/3

Embryonic carcinoma 9070/3

Yolk sac tumor 9071/3

Chorionic carcinoma 9100/3

Teratoma 9080/1

mature 9080/0

immature 9080/3

Teratoma with malignant transformation 9084/3

Mixed germ cell tumor 9085/3

3.7. Tumors of the sella turcica

Craniopharyngioma 9350/1

adamantine 9351/1 in =

papillary 9352/1 in =

Granular cell tumor 9582/0 in =

Pitucytoma 9432/1 in =

Spindle cell oncocytoma of the adenohypophysis 8291/0 in =!

3.8. Metastatic tumors hereditary tumor syndromes with

involvement of the nervous system

Neurofibromatosis type 1

Neurofibromatosis type 2

Hippel-Lindau syndrome

Tuberous sclerosis

Li-Fraumeni syndrome

Covden syndrome

Turko syndrome

Gorlin syndrome

qualities, such as infiltrative growth, cellular and nuclear pleomorphism, are considered in slightly different aspects. Particular attention should be given to other characteristics, such as the ability to metastasize both within the CNS - along the cerebrospinal fluid pathways, along the membranes, and the ability to metastasize beyond the CNS; assessment of the severity of vascular proliferation as one of the main factors of aggression of astroglial tumors, as well as the presence of necrosis - both ischemic type and specific - “geographical” or “palisade” type.

This gradation provides for 4 degrees of malignancy, indicated by Roman numerals (I degree is the most benign, and II, III and IV indicate an increasing degree of malignancy). It is prognostically significant, and such an assessment of a specific tumor is given not from a morphological assessment of this specific tumor, but based on a retrospective analysis of prognostic significant factors of many tumors of a similar structure.

Only germ cell tumors and primary CNS lymphomas are not designated according to this 4-point system.

For example, we can consider a dysembryoplastic neuroepithelial tumor, then the ICD/O code (9413/0) indicates the absolute formal-morphological benignity of the process, but it is assigned the I (lowest) gradation of the degree of malignancy of CNS tumors - G=I. According to these requirements, in the morphological report the pathologist should indicate, in addition to the oncological unit, two gradations of the degree of malignancy - according to the ICD/O and according to the 4-point system. Example of a conclusion: “... fragments of a diffusely growing glial tumor of spindle-shaped bipolar cells with Rosenthal dystrophy of fiber processes, without obvious cellular and nuclear polymorphism, are presented. No mitoses, vascular proliferation or necrosis were found. Histological picture of pilocytic astrocytoma, ICD/O code - 9421/1, I degree of malignancy (c=C".

More information about hereditary tumor syndromes:

Neurofibromatosis of the first and second types are hereditary tumor syndromes that differ in some details of oncogenesis and clinical and morphological manifestations with impaired synthesis of proteins such as merlin and schwannomin. The well-known term “Recklinghausen's disease” applies only to neurofibromatosis type 1, and bilateral acoustic neuromas are now considered a manifestation of neurofibromatosis type 2.

Hemangioblastomas are a component of Hippel-Lindau disease (VHL) in 25% of cases; The existence of spontaneous hemangioblastomas is also allowed. There is a clear indication of the cellular substrate of the tumor - stromal vacuolated cells, in the cytoplasm of which oncoprotein was identified using immunohistochemistry methods - the product of the VHL gene of the same name, responsible for oncogenesis.

Tuberous sclerosis in the central nervous system manifests itself as subependymal growths of low-grade giant cell astrocytoma. Manifestations in other organs and systems may include sebaceous adenomas of skin appendages, cardiac rhabdomyomas, and multiple renal angiomyolipomas. Synonyms that are also commonly used to refer to tuberous sclerosis are Bourneville disease, Bourneville-Pringle disease.

Li-Fraumeni syndrome is characterized by multiple primary malignant tumors of children, adolescents and young adults, including: soft tissue and skeletogenic sarcomas, breast cancer, leukemia and an increased incidence of central nervous system tumors, among which astroglial and embryonal tumors are the leaders. It is believed that the reason is mutations in the “guard” of the genome - the TP53 suppressor gene.

Cowden's disease and dysplastic cerebellar ganglion-ocytoma (Lhermitte-Duclos disease) are an autosomal dominant condition characterized by multiple hamartomas and tumors. The main manifestation in the central nervous system is dysplastic cerebellar gangliocytoma, a morphologically completely benign tumor of a two-cell subpopulation of mature neurons, histogenetically derived from Purkinje cells.

Turcot syndrome is a combination of colorectal adenomas/carcinomas with medulloblastomas or anaplastic astrocytomas/glioblastomas. Most cases of Turcot's syndrome occur within diffuse familial polyposis or congenital nonpolyposis colon carcinoma syndrome.

Gorlin syndrome is manifested primarily by multiple basal cell cutaneous carcinomas throughout the body in combination with various developmental anomalies, hamartomas, benign and malignant tumors - meningiomas, melanomas, lymphomas, lung and breast carcinomas, and ovarian dermoid tumors. A common tumor of the central nervous system that occurs as part of this syndrome is cerebellar medulloblastoma, most often of the desmoplastic histotype.

In the third and fourth editions of the WHO classification of NS tumors, some new nosological units appeared, the identification of which would have been impossible without the use of new, modern research methods (cytogenetics with the determination of chromosomal aberrations, loss of heterozygosity), as well as molecular genetics (detection of point mutations and expression some oncogenes and blocking key suppressor genes, comparative genomic hybridization, the use of biochips, etc.).

New oncology units

Cerebellar liponeurocytoma is a very rare tumor of the vermis or cerebellum, consisting of mature neurocytes and mature adipose tissue. The tumor cells have low mitotic activity, which determines its long duration and a fairly favorable prognosis with the most complete removal.

Chordoid glioma of the third ventricle is a rare, located in the anterior portion of the third ventricle, a slowly growing tumor of a peculiar structure, consisting of trabeculae of epithelioid cells separated by mucinous stroma. Characterized by dense lymphoplasmacytic infiltration of the stroma, often even with the presence of Roussel's bodies. Tumor cells have a low proliferative potential, and the prognosis for subtotal removal is quite favorable, although the tumor location is inaccessible, which necessitates a traumatic approach and removal.

There is no such nosological form as “primitive polar spongioblastoma,” which was identified by most authors since the 1920s. As rightly pointed out in the 1990s. domestic neuropathohistologist A.G. Korshunov, this is one of the morphological variants of hemispheric neuroblastoma.

Also, taking into account the localization and biological behavior, such a nosological unit as “pleomorphic xanthoastrocytoma” was identified. This tumor, in which there is pronounced pleomorphism, the presence of giant and multinucleated cells and xanthoma cells; their cytoplasm is often vacuolated. It occurs predominantly in young people and has a convexital localization. It is characterized by slow growth, rare relapses, and has a fairly good prognosis (five-year disease-free survival over 75% and ten-year - 63%).

Pilomyxoid astrocytoma is a variant of pilocytic astrocytoma in children under one year of age, but with a more aggressive course. Under microscopy, bipolar tumor cells are found in it, enclosed in a myxoid matrix; Around vessels, cells often form angiocentric structures. Unlike pilocytic astrocytoma, it has a higher proliferative activity; in the cytoplasm and cell processes there are no signs of Rosenthal dystrophy.

Angiocentric glioma is a rare, slow-growing neuroepithelial tumor with a predominant localization in the frontal, temporal or parietal lobe; as a rule, adjacent to the bark. The tumor is epileptogenic, which is its characteristic feature (chronic and difficult to treat). Most patients experience epileptic seizures long before the tumor is detected (an average of 7 years). Morphologically, the tumor is built from monomorphic cells

current, which form peculiar, so-called “angiocentric” structures around vessels of various sizes. They resemble perivascular ependymal rosettes. Their similarity with ependymomas does not end there - they exhibit immunomorphological and electron microscopic signs of ependymal differentiation, which may indicate the probable histogenesis of the tumor.

Papillary glioneuronal tumor is a rare, usually well-circumscribed, solid-cystic tumor of the cerebral hemispheres, most often in the parietal lobe. Histologically, it consists of closely packed papillae and pseudopapillae, covered by a single layer of cuboidal glial cells with focal accumulations of neurons. The stroma contains hyalinized vessels. The prognosis is favorable; after removal, the tumor rarely recurs.

Rosette-forming papillary glioneuronal tumor is a very rare tumor, the characteristic feature of which is its localization in the midline - the fourth ventricle, trunk, aqueduct of Sylvius, cerebellar vermis, pineal gland. The histological structure is biphasic - the neuronal component forms multiple rosettes, the glial component may even look like a pilocytic astrocytoma. In cases of resectable tumor, the prognosis is favorable.

Extraventricular neurocytoma is morphologically identical to central neurocytoma, but with such localization microscopically it is difficult to distinguish it from oligodendroglioma (small round cells with clear cytoplasm, forming honeycomb-like structures).

Atypical papilloma of the choroid plexus - differs from benign papilloma by increased cellularity, mitotic activity, areas of solidification and the appearance of necrosis.

Pituycytoma is a very rare solid, encapsulated tumor of the neurohypophysis or infundibulum of the hypothalamus, which was previously called "granular cell tumor", "posterior pituitary astrocytoma" or "infundibuloma". Histologically, these are tumors of elongated cells with a tuft or moiré type of structure. The tumor is subject to surgical removal, after which it does not recur; there are no descriptions of malignant transformation or metastasis.

Spindle cell oncocytoma of the adenohypophysis is an extremely rare benign tumor of oncocytic/epithelioid cells, accounting for 0.4% of all tumors of the sella turcica. Despite the spindle cell configuration, its cytoplasm contains many enlarged, dilated mitochondria, which indicates oncocytic transformation. Cases of recurrence of non-radically removed tumors with increased mitotic activity and necrosis have been described.

A rhabdoid tumor with a hereditary predisposition is a highly aggressive tumor, the cells of which have a wide cytoplasm with a nucleus displaced to the periphery, very reminiscent of rhabdomyoblasts. Large inclusions are often found in the cytoplasm, which give a strong vimentin stain. The gene responsible for malignant transformation is located in the second codon of the long arm of the 22nd pair of chromosomes. In addition to a tumor in the central nervous system, synchronous

to detect a tumor of a similar structure in the kidney, lung or soft tissues.

Spinal paraganglioma (paraganglioma of the terminal filum of the cauda equina) is a rather rare, usually encapsulated tumor, with a characteristic endocrine cell alveolar-lobular type of structure, histologically similar to sympathetic paraganglioma (pheochromocytoma). Consists of two types of cells - polygonal endocrine and elongated supporting cells. Men get sick more often, the average age of patients is 46 years.

I would like to emphasize that earlier, when considering tumors of the pineal gland (pinealomas), including in the domestic literature, tumors completely different in histogenesis were confused - true pinealomas and primary germinomas of the central nervous system, which were called “two-cell type pinealomas.” In pineal cells, as in cells of the normal pineal gland, photoreceptor differentiation is detected, and the morphology of pineal germinoma is indistinguishable from the morphology of testicular seminoma and ovarian dysgerminoma; in the blood serum of these patients the level of oncofetal proteins is increased. These are tumors with completely different course of the disease, treatment protocols, monitoring of cure and prognosis. To pinealomas of varying degrees of malignancy is added a papillary tumor of the pineal gland, which has ependymal differentiation, often recurs and has a poor prognosis.

Ependymomas - the list of ependymomas of the second degree of malignancy has been expanded - they are divided into four types, and anaplastic ependymoma (third degree of malignancy). Ependymomas of the second degree of malignancy are distinguished by cellular phenotype - cellular, papillary, clear cell and tanycytic ependymomas (Greek lapuov - elongated).

Meningiomas - specified by type; Nine variants of typical meningiomas were identified. Chordoid and clear cell meningiomas are classified as atypical, rhabdoid and papillary meningiomas are classified as anaplastic. Thirdly, the group of meningiomas was significantly changed, which were divided into three groups according to the degree of malignancy (typical, atypical and anaplastic). The following were added to typical meningiomas: microcystic, secretory, clear cell, chordoid, metaplastic, rich in lymphoplasmacytic cells.

From the group of meningiomas in general, hemangioblastic and hemangiopericytic variants were isolated, which were transformed into mesenchymal tumors of the membranes. Although extrathecal hemangiopericytomas are now generally classified as tumors of the group of solitary fibrous tumors, hemangiopericytoma of the membranes not only retained its historical name, but its “anaplastic” variant was also identified.

A retrospective analysis of tumors with immunophenotyping, previously interpreted as “meningeal sarcomatosis”, showed that these were metastases of cancer, lymphomas, gliomas and tumors of the Ewing sarcoma family. The latter are included in the group of mesenchymal non-meningothelial tumors of the membranes.

When evaluating the classifications of the third and fourth revisions, it should be recognized. How do they compare favorably?

differ from previous editions by increasing the list of nosological forms due to a retrospective analysis of some tumors in comparison with long-term results. This approach made it possible to identify some nosological forms with a relatively favorable prognosis and a less stringent adjuvant protocol.

LITERATURE

1. Matsko D.E. Modern histological classifications of tumors of the central nervous system. Current issues in oncomorphology / Ed. N.M. Anichkova, A.E. Kolosova. - St. Petersburg-Kirov, 1996. - P.81-91.

2. Matsko D.E., Korshunov A.G. Atlas of tumors of the central nervous system. - St. Petersburg, 1998.

3. Khominsky B. S. Histological diagnosis of tumors of the central nervous system. - M., 1969.

4. Zulkh K.D. Histological classification of tumors

chemoradiation treatment. Modern methods of molecular biological research of tumors provide new facts, the understanding of which is the basis for identifying new oncological units

and, undoubtedly, the classification will be improved and revised.

central nervous system. - M.: 1983.

5. Kleihues P., Burger P. C, Scheithauer B. W. Histologic classification of tumors of the central nervous system. - New York: Springer-Verlag, 1993.

6. Pathology and Genetics of Tumors of the Nervous System / Eds. P Kleihues, W.K. Cavenee. - Lyon: IARC Press, 2000.

7. Zulch K.J. Histologic Typing of Tumors of the Central Nervous System. - Geneva, 1979.

8. WHO Classification of Tumors of the Central Nervous System (2007) / Eds. D.N. Louis, H. Ohgaki, O.D. Whistler, W. Cavenee. - Geneva: WHO Press, 2007. - R. 16-172.

Correspondence address:

664079, Irkutsk, Mr. Yubileiny, 100, PO Box No. 35, Yuri Klimentievich Batoroev - assistant of the Department of Oncology, ISIUV, e-mail: [email protected]

© PINSKY S.B., Dvornichenko V.V., REPETA O.R. - 2009

METASTATIC TUMORS OF THE THYROID GLAND

S.B. Pinsky. V.V. Dvornichenko. O.R. Repeta

(Irkutsk State Medical University Rector - MD, Prof. I.V. Malov, Department of General Surgery with a course of urology, Head - MD, Prof. S.B. Pinsky; Institute for Advanced Medical Studies , Rector - Doctor of Medical Sciences, Prof. V.V. Shprakh, Department of Oncology, Head - Doctor of Medical Sciences, Prof. V.V.

Resume. The article presents literature data and an analysis of our own 10 observations of metastasis of malignant tumors of various morphogenesis to the thyroid gland. Data on their frequency is provided. features of the clinical course. difficulties and errors in diagnosis and choice of treatment method. Particular attention is paid to metastasis of clear cell renal cancer. difficulties in their diagnosis and choice of treatment tactics, unsatisfactory prognosis.

Key words: thyroid gland. metastatic cancer. clear cell kidney cancer.

METASTATIC TUMORS OF THE THYROID GLAND

S.B. Pinskiy, V.V. Dvornichenko, O.R. Repeta (Irkutsk State Medical University, Irkutsk State Institute for Medical Advanced Studies)

Summary. The report contains data from the literature and our own analysis of 10 cases of malignant tumor metastatic spreading to the thyroid gland. The data on frequency, clinical features, difficulties and mistakes in making diagnoses and choosing method of treatment are given. Special attention is paid to the metastases of kidney cancer, difficulties in diagnostics, choice of treatment and poor prognosis.

Key words: thyroid gland, metastatic carcinoma, clear cell renal carcinoma.

In the problem of malignant tumors of the thyroid gland, the question of the synchronous and metachronous development of various neoplasms of the thyroid gland and tumors of other localizations remains important. Newly detected tumor formations after treatment of malignant neoplasms are usually a consequence of progression of the underlying disease. In such observations, first of all, it is necessary to exclude the metastatic nature of the thyroid tumor. Metachronous tumors of the second localization require a differentiated approach in diagnosis and choice of treatment tactics. Timely detection of isolated metastases in the thyroid gland with a verified primary tumor and the absence of other metastatic foci creates the prerequisites for their surgical removal, improving survival and quality of life. At the same time, to this day there are diagnostic difficulties both in recognizing metastatic tumors of the thyroid gland and in identifying the primary tumor in the presence of metastasis in the thyroid gland.

The variability of clinical manifestations makes it difficult to timely diagnose metastatic thyroid tumors. In most published cases, metastatic thyroid tumors were diagnosed as nodular goiter or primary thyroid cancer. Even in those observations in which the primary tumor was recognized, metastatic tumors were often diagnosed as primary diseases of the thyroid gland, and only histological examination of the surgical material made it possible to verify the true nature of the neoplasm.

The literature provides very contradictory information about the frequency of metastasis of malignant tumors to the thyroid gland, both according to clinical observations and autopsy results. J. Moyeshep et al. (1956) reported autopsy data from 467 patients with various malignant tumors, 18 (3.8%) of whom were found to have metastatic thyroid tumors. K. 8Ytaoka et al. (1962) based on materials from 1999 autopsies revealed me-