Hemangioblastomas are vascular neoplasms that occur in the central nervous system. In the majority of cases, the disease presents in young adults. In principle, hemangioblastomas are a benign form of tumor. The tumor is usually located in the cerebellum.
What is a hemangioblastoma?
In principle, a hemangioblastoma is a special tumor that has a large number of vessels. In this regard, hemangioblastoma occurs in most cases in the area of the central nervous system. In addition, there is a possibility of hemangioblastoma arising in the tissues of soft tissues. According to the World Health Organization, hemangioblastomas are considered benign tumors. In this regard, they are categorized as grade 1 tumors of the central nervous system. In some cases, hemangioblastomas appear together with the so-called Hippel-Lindau syndrome. In addition, sporadic occurrence of the tumors is also possible. Frequently, hemangioblastomas show up in the area of the brain stem, cerebellum or in the medulla of the back. In rare cases, the tumors also appear in the area of the cerebrum. In addition, it is possible for hemangioblastomas to form on the retina of the human eye. In this case, they are often called retinal angiomas. However, this name is not correct. Basically, about ten percent of all tumors localized in the posterior region of the cranial fossa are hemangioblastomas. In the majority of cases, patients are between 20 and 40 years old at the time of the disease. The disease occurs more often in men than in women. Hemangioblastomas most frequently form in the cerebellar hemispheres or the cerebellar vermis. Ten percent of all hemangioblastomas develop in the medulla of the back, and only three percent in the brainstem.
Causes
Currently, the exact causes of hemangioblastoma formation are still mostly unclear. Basically, the tumors arise from the so-called pia mater as well as various pathologic capillaries. Why they transform into hemangioblastomas has not yet been sufficiently researched. In principle, approximately 80 percent of all hemangioblastomas occur sporadically, while about 20 percent occur together with Hippel-Lindau syndrome.
Symptoms, complaints, and signs
Hemangioblastomas cause a variety of symptoms, depending primarily on their location. For example, cerebral symptoms such as ataxia or speech disorders are possible. Sometimes there is also a root compression syndrome or a spinal cord syndrome. Some hemangioblastomas produce the substance erythropoietin. This substance causes red blood cells to proliferate (medical term polycythemia). From a macroscopic point of view, the tumor appears to be 60 percent cystic and 40 percent solid. The tumor is round in shape and has a yellow coloration due to the high proportion of fatty tissue. On microscopic examination, capillaries with thin walls are visible. Hyperplastic endothelial cells can also be seen. Pericytes are enclosed by specialized stromal cells. Hemangioblastomas have a large amount of the substance reticulin. Mitoses do not occur in the setting of hemangioblastomas, but hemorrhage, necrosis, and calcification are possible in rare cases. Hemangioblastomas in the spinal cord area often occur together with a fluid sac. This is also known as a syrinx and causes numerous symptoms. If the hemangioblastoma damages the cerebellum, symptoms may include dysmetria, gait ataxia, vertigo, and dysdiadochokinesia. If the hemangioblastoma is located in the brainstem, cranial nerve deficits often result.
Diagnosis and disease progression
With regard to the diagnosis of hemangioblastoma, various examination methods come into consideration, the use of which is decided by the treating physician. In principle, imaging examination methods are of greatest importance in establishing the diagnosis of hemangioblastomas. In radiology, hemangioblastomas usually present as space-occupying lesions that absorb administered contrast agents and are characterized by a pseudocystic shape. When computed tomography or magnetic resonance imaging is performed, cystic hypodense space-occupying lesions are seen in 60 percent of cases. Only 40 percent of all hemangioblastomas are solid in shape.As part of the differential diagnosis, renal cell carcinoma should be considered. This is because the corresponding metastases may resemble hemangioblastoma. However, histologic examinations can be used to prevent confusion.
Complications
Various symptoms may result from hemangioblastoma. Usually, the symptoms and further course of the disease depend greatly on the affected area. In most cases, however, there are disturbances in coordination, concentration, and also speech disorders. These can negatively influence the patient’s everyday life. Furthermore, those affected suffer from hemorrhages and calcifications of the vessels. If the tumor penetrates into the cerebellum, various limitations in the cognitive processes may occur. In this case, dizziness or gait disorders often occur. As the disease progresses, cranial nerves may also fail without treatment, resulting in restricted movement or paralysis. The patient’s quality of life is reduced by the hemangioblastoma. Usually, treatment of hemangioblastoma does not lead to further complications. The tumor can be easily removed in most cases. Complications can arise if the removal of the tumor is done late and thus the tumor has already affected or damaged other regions. In this case, life expectancy may be reduced. However, if treatment is successful, there is no change in life expectancy.
When should you see a doctor?
Immediate treatment must always be given for hemangioblastoma to prevent further complications and further spread of the tumor. If treatment is not initiated, the person affected by hemangioblastoma may die in the worst case. A doctor should be consulted if speech disorders occur without a specific reason. Affected individuals may also suffer from impaired sensitivity or various sensory disturbances, which may also be indicative of hemangioblastoma. Frequently, hemorrhages also occur in the skin. Furthermore, dizzy spells or gait disturbances may indicate the disease and should always be investigated if they persist over a longer period of time. However, the severity of the symptoms can vary greatly. In the first instance, a pediatrician or general practitioner can be consulted for these complaints. With the help of various examinations, the hemangioblastoma can then be diagnosed. Whether direct removal is necessary, however, will be decided depending on the extent of the tumor.
Treatment and therapy
Hemangioblastomas are relatively easy to treat, depending on the location and extent of the tumor. Removal of the tumor is usually the treatment of choice. The hemangioblastoma is removed as completely as possible in a surgical procedure. It is important that the cyst wall is also completely removed. Subsequently, the prognosis is relatively positive. This is especially the case if it is a cellular subtype of hemangioblastoma. Sometimes it is difficult to distinguish hemangioblastoma from a secondary tumor of Hippel-Lindau disease. However, if the tumor is completely resected, the prognosis is relatively favorable.
Prevention
According to the current state of knowledge of medical and pharmacological research, no effective measures for the prevention of hemangioblastomas are known yet. This is because the causes for the formation of this type of tumor are also still largely unexplained. For this reason, timely diagnosis as well as therapy of hemangioblastoma play the most important role. In case of characteristic complaints and symptoms, a suitable specialist should be consulted as soon as possible.
Follow-up
The treatment of a cancer is always followed by aftercare. This is because there is a risk of a new tumor developing in the same place. Doctors perform follow-up care at least quarterly during the first year of diagnosis. After that, the rhythm expands. If there is still no new growth in the fifth year, a one-year check-up is then due. The patient receives detailed information on this. Follow-up care often takes place in the clinic where the initial procedure was performed. Imaging techniques such as MRI and CT are used to diagnose hemangioblastoma. In rare cases, the disease requires long-term follow-up care because secondary damage persists.These can be treated in various therapies. A rehabilitation program promises rapid success. In this, experts from various fields are available and can specifically adjust the patient for everyday life. Suitable medication can also be prescribed in this way. Neurological problems sometimes require fundamental changes in life. This can cause psychological stress. Psychotherapy can then help. It should be noted, however, that a hemangioblastoma is a benign tumor. Consequential damage that has an impact on everyday life is an exception.
What you can do yourself
There are no self-help options available to the affected person with a hemangioblastoma. In any case, this tumor must be treated by a physician, and surgery or radiation is usually necessary. Since hemangioblastoma has a negative effect on the general condition of the affected person, the patient should rest and not expose the body to unnecessary stress. Bed rest and relaxation techniques can have a positive effect on the disease. Furthermore, patients need help and support from friends and family. Loving care also has a positive effect on the course of the disease. Possible psychological complaints can be addressed with the help of discussions. Children should also be fully informed about the possible course of this disease. In many cases, discussions with other affected persons or, in the case of severe mental stress, discussions with a therapist also help, whereby even an exchange of information can be very helpful. Since an early diagnosis has a very positive effect on the course of the disease, an examination should be carried out at the first signs. Regular examinations are also necessary after treatment in order to detect and treat possible further tumors at an early stage.
