Hemangioblastoma

Hemangioblastoma
A hemangioblastoma is a histologically benign tumor that consists of blood vessels. It is quite rare. Hemangioblastoma occurs most often in the cerebellum, less often in the brain stem and in the spinal cord.

The content of the article:
Causes of hemangioblastoma
Classification of hemangioblastoma
Symptoms of hemangioblastoma
Diagnosis of hemangioblastoma
Treatment of hemangioblastoma
Irradiation of hemangioblastoma
Prognosis of hemangioblastoma
Hemangioblastoma
This tumor can form cysts and solid components. These are isolated formations that occur in middle-aged people (35-36 years old). Hemangioblastoma has an unclear histological origin, but presumably this formation originates from mesenchymal cells, vascular origin or hematopoietic stem cells. Given the macroscopic structure of the formation, hemangioblastomas are soft-tissue (capsule-shaped node of purple color) and large smooth-walled cysts (a cyst containing a yellowish liquid).

Causes of hemangioblastoma
This tumor can occur independently or in people with Hippel-Lindau disease (cerebroretinal angiomatosis), transmitted by an autosomal dominant method. In this case, the formation often has a malignant character.

A possible cause of the manifestation of this disease is a mutation of the third chromosome, the autosomal dominant VHL gene, the pathology of which can lead to the appearance of not only hemangioblastoma, but also to many other benign or malignant formations. This genetic pathology can be inherited from parents or carriers of a mutating gene.

Factors contributing to the appearance of hemangioblastoma are carcinogenic:

exposure to ionizing radiation;
viral infections affecting the central nervous system (herpes, adenovirus);
the impact of vinyl chloride, benzene, various types of resins, etc.;
the influence of adverse environmental factors;
hereditary diseases.
Classification of hemangioblastoma
Hemangioblastomas are of two types — cystic and solid. Solid formations consist of tumor cells, cystic hemangioblastomas are a solid component of a small size, soldered to the cystic part of the tumor, much larger in size. This tumor, if localized in the brain, usually grows during its development and creates increased intracranial pressure.

Taking into account the macroscopic structure of the formation, hemangioblastomas are of two types: soft-tissue (they are a capsule-shaped node of purple color), they are more common than other varieties of hemangioblastomas and large smooth-walled cysts (a cyst containing a transparent yellowish liquid).

In some cases, there are also mixed types of this formation — a hemangioblastoma is a solid node, inside which there are multiple cystic formations.

Based on the histological structure of hemangioblastomas, several types of it were identified:

Transitional: consists of approximately an equal number of thin-walled capillaries and stromal cells, which contain a large amount of fat.
Juvenile: formed from a large and dense cluster of thin-walled capillaries.
Pure cell: includes many xanthochromic cells that are attached to vessels affected by pathologies.
Symptoms of hemangioblastoma
The clinical picture and symptoms of this disease depend, first of all, on the localization of the formation and its size. They often include general cerebral symptoms that are provoked by disorders of the outflow of cerebrospinal fluid, as well as progressive hydrocephalus. Hemangioblastoma, acting as an intracerebral tumor, is quite rare. In most cases, this disease affects the upper tissues of the cerebellum and very rarely its internal structures (worm).

With the progression of hemangioblastoma , the following symptoms may occur:

Severe headache (more often in the occipital area), which does not stop even when taking painkillers or vasodilators. Pain syndrome has a paroxysmal character, can be caused by physical exertion, nervous overstrain, rapid change of body posture;
Frequent dizziness;
Disorders of the vestibular apparatus (dynamic ataxia);
Periodic nausea causing vomiting (a state of “stagnant labyrinth”);
Weakness in the limbs and loss of motor function (inability to repeat the Romberg pose);
Sensitivity disorders in different parts of the body;
Disorders of the bladder and intestines;
Complex neurological disorders;
Mental disorders at the late stages of the development of education (increased emotionality, sometimes aggressiveness, irritation);
Disorders of the visual and auditory nerves;
Change in handwriting or gait: deviation of the whole body — lateropulsion, staggering when walking, violation of synchronicity of movements may be observed;
Decerebral rigidity — paroxysmal uncontrolled muscle tension;
Unnatural tilt of the head (the patient finds a head position in which the pain syndrome or other signs of the disease become less painful and uncomfortable);
Muscular hypotension, nystagmus.
Diagnosis of hemangioblastoma
The diagnosis of hemangioblastoma is difficult, because at the initial stages it is almost impossible to determine the cerebellar syndrome. This neoplasm has a similar clinical picture with a number of other diseases associated with neurological abnormalities or oncological formations of a different kind.

When diagnosing this disease , the following methods are most often used:

Computed tomography: makes it possible to confirm the presence and localization of a tumor node or cystic formation.
Magnetic resonance imaging is a procedure for the introduction of special drugs that allows you to visualize the formation. The MRI procedure will be especially effective for cystic formations, since they accumulate contrast agent better.
Cerebral angiography: allows you to examine the area of vessels that support blood circulation in the neoplasm. As a result of this procedure, veins and arteries, arteriovenous shunts that have been pathologically altered are revealed.
Spinal angiography — reveals the localization of the tumor node, allows you to examine the large arteries adjacent to it.
For the final determination of the presence of hemangioblastoma and the diagnosis of its specific type, a histological examination of the tumor particle is necessary. To do this, a stereotactic brain biopsy is performed.

Treatment of hemangioblastoma
Most often, a hemangioblastoma is a benign uninvested tumor, so surgical intervention in most cases is sufficient for treatment. These neoplasms are well limited from the tissues of the brain or spinal cord, do not grow and do not metastasize (therefore, chemotherapy in these cases is ineffective), which makes it possible to remove them qualitatively.

Surgeons, first of all, should analyze the localization of the tumor, the fact of the presence or absence of cystic formation, its nature, the development of hydrocephalus, the presence of edema around the formation, as well as the density of nearby nerve structures. After that, you can decide on the adequacy of a particular method of surgical intervention.

The bright color of the tumor often makes it easy to determine its localization and visualize the neoplasm. If there is a cyst, it can be drained with a syringe (decompression process). This procedure allows you to more clearly visualize the line of demarcation between the upper tissues of the spinal cord or brain and the tumor body.

A dangerous moment during the operation is the formation of a fossa after the removal of the tumor. The danger is that there may be bleeding from the walls of this fossa. The timely reaction of the neurosurgeon and the application of cotton balls will be able to stop the blood within a few minutes.

Like any other operation, removal of hemangioblastoma is associated with certain risks, for example, infection, complications after anesthesia.

Radiosurgical operation
An alternative method of traditional surgery is the “Cyber—knife” equipment and radiosurgical surgery with its help. This procedure largely benefits from traditional methods of surgery. It does not require anesthesia and is painless, allows you to remove neoplasms even in inoperable patients. Complications occur much less frequently in comparison with a conventional operation, the intervention is not complicated by a long rehabilitation period. It is used in cases of localization of the tumor in places that are difficult to access for traditional methods of surgery.

Irradiation of hemangioblastoma
In some cases, after the removal of the tumor, there is a need for radiation therapy. The procedure is the irradiation of a tumor with a high degree of radiation to stop growth or even reduce it.

Radioactive radiation disrupts blood circulation in the tumor body, prevents the fact of erythrocytosis. The method of radiation therapy is not able to completely remove the tumor and is used in case of inoperable patient.

Prognosis of hemangioblastoma
Timely medical treatment, high-quality diagnosis and radical treatment can give good prognoses for hemangioblastoma. About 80% of cases of this formation have favorable prognoses. Postoperative relapse can occur only with partial removal of the tumor, and the prognosis may be less favorable against the background of the presence of Hippel-Lindau disease, since there remains a risk of new formations of this type in different areas of the central nervous system, despite the removal of the focus. With sporadic tumors, provided they are completely surgically removed, repeated neoplasms no longer occur.

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