Hemangioblastomas

Hemangioblastoma is a benign tumor that consists of blood vessels. It is localized within the central nervous system and most often occurs in the cerebellum and in some cases in the spinal cord or brain.

The disease does not have a clear histological origin and is manifested by a violation of the normal outflow of cerebrospinal fluid, damage to the cerebellum.

In the general statistics of neoplasms in the cranium, hemangioblastoma is diagnosed in 2% of all cases. People at the age of 30-65 are at risk of developing a tumor. Men are more likely to suffer from the disease than women.

Often the cause of hemangioblastoma is Hippel-Lindau disease. The disease is inherited in an autosomal dominant manner and is characterized by multiple congenital cysts of internal organs. Hemangioblastoma provoked by Hippel-Lindau disease is most prone to recurrence in other parts of the central nervous system.

Types of hemangioblastoma

Hemangioblastoma is divided into two main categories: macroscopic and microscopic.

Types of macroscopic hemangioblastoma:

  • Solid. The neoplasm consists of tumor cells combined into one capsular node. The tumor is soft, dark red in color. This type of hemangioblastoma is diagnosed in 70% of cases. A bulky tumor of the spinal cord or brain puts pressure on the tissues, provoking neurological symptoms;
  • Cystic. The tumor is a smooth-walled cyst, which has formations of a solid component on the walls;
  • Mixed. The neoplasm looks like a solid hemangioblastoma, but there are multiple cysts inside it.

Types of microscopic hemangioblastoma:

  • Juvenile. Tumor with closely spaced capillary vessels;
  • Pure cell. The neoplasm consists of cells that are located on the blood vessels;
  • Transitional. Education in the same proportion consists of capillary vessels and stromal cells.

Causes of hemangioblastoma

One of the main reasons for hemangioblastoma is hereditary Hippel-Lindau disease. Phakomatosis appears due to a genetic mutation of the third chromosome. The consequence of the disease is the development of multiple tumors of the spinal cord, kidney and pancreas.

The main risk factors:

  • Previous infections affecting the central nervous system;
  • Influence of ionizing radiation;
  • Increased insolation;
  • Working conditions associated with production carcinogenic factors (vinyl chloride, resins);
  • Living in an unfavorable ecological environment.

Symptoms

The symptomatology of the disease corresponds to the location of the tumor.

There are 3 subtypes of signs of hemangioblastoma:

  • General cerebral;
  • Cerebellar;
  • Remote.

Severe headache is characteristic for the cerebral subtype of the disease. Discomfort from spasm which is localized in the back of the head cannot be eliminated with spasm-prayer drugs and analgesics. Pain syndrome can be accompanied by nausea and frequent urge to vomit.

A common symptom of hemangioblastoma is optic nerve dysfunction. Vision problems force the patient to go to an ophthalmological clinic, which complicates the diagnosis of the disease. Also, the blurring of symptoms and the complexity of diagnosis is affected by vestibular ataxia, the signs of which are similar to inflammation of the inner ear with otitis media.

Cerebellar type features include cerebellar ataxia. Pathology consists of a complex of specific disorders of static and dynamic motor skills, disorders of coordination of movements.

When examined by a neurologist, megalography (a change in the usual handwriting to a more sweeping and large one), as well as dysmetria (non-ordered movements with a wide girth) can be diagnosed.

The danger of cerebellar tumors is their rapid growth. A large tumor puts pressure on the structures of the brain and provokes abnormalities in the functions. Thus, the disease is classified as a bilateral lesion.

Long-term symptoms include:

  • Convulsions;
  • Disruption of bowel function;
  • Paralysis of the cranial nerves;
  • Violation of sensitivity in different areas of the body;
  • Bladder dysfunction;
  • Neurological disorders (asynergies);
  • Violation of the musculoskeletal system.

In the later stages of the disease, mental disorders can occur. The patient becomes irritable and aggressive or, conversely, may experience apathy.

Diagnostics

Due to the versatility of symptoms, it is rather difficult to diagnose cerebellar hemangioblastoma. The consultation of an ophthalmologist is of particular importance. During the examination, the specialist discovers stagnant optic discs. This is the key reason to suspect oncology.

Basic diagnostic methods:

  • Electroencephalography. EEG is used to assess the electrical activity of brain structures affected by the disease;
  • Rheoencephalography. The study helps to determine the condition of the blood vessels and the outflow of blood in the lesion;
  • Spinal angiography. A research method in which a probe is inserted into all the vessels that feed the spinal cord;
  • Echoencephalography. A non-invasive ultrasound diagnostic method that allows determining pathological processes and deformations in the structure of the brain;
  • MRI. Magnetic resonance imaging is the most effective method for diagnosing hemangioblastoma. Scanning the structures of the brain in a three-dimensional plane allows detecting an intracranial neoplasm, assess the size of the tumor and see the vessels supplying the tumor;
  • Biopsy. To determine the type and nature of the neoplasm, it is necessary to collect particles of the affected tissue for histological examination. A stereotaxic biopsy is needed to obtain a sample. In some cases, particles of tumor tissue are collected directly during surgery (intraoperative biopsy).

Treatment

Hemangioblastoma is treated with several methods: surgery, radiation therapy, and chemotherapy. In difficult clinical cases, specialists draw up a plan for the treatment of hemangioblastoma after surgery.

Radiation therapy

Radiation therapy for hemangioblastoma is mainly aimed at slowing tumor growth but is also used for patients for whom surgery is contraindicated for general health reasons. Another indication for the method of radiation therapy is the inoperability of hemangioblastoma due to its dangerous location.

Chemotherapy

Chemotherapy means treatment with special medications that negatively affect the tumor, slowing down its growth and development. The drugs are administered intrathecally (after puncture of the dura mater). The intrathecal method allows the most effective effect on neoplasm.

Chemotherapy is used both as an independent treatment method and in combination with radiation therapy for maximum efficiency.

Surgical treatment

Surgical treatment provides for the complete excision of the neoplasm using a craniotomy, as well as the removal of the occipital part of the bone and resection of the arch of 1 cervical vertebra. Due to modern neuronavigation systems, it is possible to influence the affected area accurately without contacting the surrounding tissues. In the case of the diagnosis of cystic macroscopic hemangioblastoma, the internal substance of the cyst is aspirated. Surgical removal of the tumor is prescribed if indicated and based on the results of diagnostics of the size, structure and localization of the tumor. In inoperable clinical situations, non-radical excision of tumor tissue can be performed to improve the patient’s condition.

Prognosis

Hemangioblastoma of the brain is a benign tumor, which is not prone to recurrence during cardinal excision. A favorable prognosis is observed in more than 80% of cases.