According to abbreviationfinder, Hemangioblastomas are vascular neoplasms that occur in the central nervous system. The disease appears in the majority of cases in young adults. Basically, hemangioblastomas are a benign form of tumors. The tumor is usually located in the cerebellum.
What is a hemangioblastoma?
In radiology, hemangioblastomas usually show up as masses that take up administered contrast media and are characterized by a pseudocystic shape.
In principle, a hemangioblastoma is a special tumor that has a large number of vessels. In most cases, hemangioblastoma occurs in the area of the central nervous system. In addition, there is a possibility that the hemangioblastoma arises in the tissue of soft tissues.
According to the World Health Organization, hemangioblastomas are benign tumors. They are classified as grade 1 central nervous system tumors. In some cases, hemangioblastomas appear together with the so-called Hippel-Lindau syndrome. A sporadic occurrence of the tumors is also possible.
Hemangioblastomas often appear in the brainstem, cerebellum, or spinal cord. 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. They are often called retinal angiomas.
However, this designation is incorrect. Basically, around ten percent of all tumors located in the rear area of the cranial fossa are hemangioblastomas. In the majority of cases, the 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 form most frequently in the area of the cerebellar hemispheres or the cerebellar vermis. Ten percent of all hemangioblastomas arise in the spinal cord, only three percent in the brainstem.
Causes
Currently, the exact causes for the formation of hemangioblastomas are still largely unclear. Basically, the tumors arise from the so-called pia mater and various pathological capillaries. Why they transform into hemangioblastomas has not yet been adequately researched. In principle, about 80 percent of all hemangioblastomas occur sporadically, while about 20 percent occur together with the Hippel-Lindau syndrome.
Symptoms, Ailments & Signs
Hemangioblastomas cause various symptoms, which primarily depend on the respective localization. For example, cerebral symptoms such as ataxia or speech disorders are possible. Sometimes root compression syndrome or spinal paraplegia may also occur.
Some hemangioblastomas produce the substance erythropoietin. This substance causes red blood cells to multiply (medical term polycythemia). Macroscopically, the tumor is 60 percent cystic and 40 percent solid. The tumor is round in shape and yellow in color 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 surrounded by special stromal cells. Hemangioblastomas contain a large proportion of the substance reticulin. Although mitoses do not occur in hemangioblastomas, bleeding, necrosis and calcifications are possible in rare cases.
Hemangioblastomas in the area of the spinal cord often occur together with a fluid sac. This is also known as the syrinx and causes numerous symptoms. If the hemangioblastoma damages the cerebellum, symptoms such as dysmetria, gait ataxia, dizziness and dysdiadochokinesis may occur. If the hemangioblastoma is in the brainstem, failures of cranial nerves are often the result.
Diagnosis & course of disease
With regard to the diagnosis of hemangioblastomas, various examination procedures can be considered, and the doctor in charge decides how to use them. In principle, imaging examination methods are of the greatest importance for the diagnosis of hemangioblastomas.
In radiology, hemangioblastomas usually show up as masses that take up administered contrast media and are characterized by a pseudocystic shape. When computer tomography or magnetic resonance imaging is performed, cystic, low-density masses are found in 60 percent of cases. Only 40 percent of all hemangioblastomas are solid.
A renal cell carcinoma should be considered in the differential diagnosis. Because the corresponding metastases may resemble a hemangioblastoma. However, with the help of histological examinations, a mix-up can be avoided.
Complications
A hemangioblastoma can cause a variety of symptoms. As a rule, the symptoms and the further course of the disease depend heavily on the affected area. In most cases, however, there are disturbances in coordination, concentration and also speech disorders. These can have a negative impact on the patient’s everyday life.
Those affected also suffer from bleeding and calcification of the vessels. If the tumor invades the cerebellum, various impairments in cognitive processes can occur. This often causes dizziness or gait disturbances. As the disease progresses, cranial nerves can also fail without treatment, leading to restricted movement or paralysis. The patient’s quality of life is reduced by the hemangioblastoma.
As a rule, the treatment of hemangioblastoma does not lead to further complications. In most cases, the tumor can be easily removed. Complications can arise if the tumor is removed late and the tumor has already affected or damaged other regions. In this case, the life expectancy can be reduced. If the treatment is successful, however, there is no change in life expectancy.
When should you go to the doctor?
In the case of a hemangioblastoma, immediate treatment must always be carried out in order to prevent further complications and the further spread of the tumor. If no treatment is initiated, the patient can die from hemangioblastoma in the worst case. A doctor should be consulted if speech disorders occur without a specific reason.
Those affected can also suffer from impaired sensitivity or various sensory disturbances, which can also indicate hemangioblastoma. Bleeding in the skin often occurs. Furthermore, dizzy spells or gait disturbances can indicate the disease and should always be examined if they persist over a longer period of time. However, the symptoms can vary greatly.
In the first place, a pediatrician or a general practitioner can be consulted for these symptoms. With the help of various examinations, the hemangioblastoma can then be diagnosed. However, whether a direct removal is necessary is decided depending on the severity of the tumor.
Treatment & Therapy
Depending on the location and severity of the tumor, hemangioblastomas can be treated relatively well. The method of choice is usually the removal of the tumor. The hemangioblastoma is removed as completely as possible during a surgical procedure. It is important that the cyst wall is also completely removed.
After that, the prognosis is relatively positive. This is especially the case when it comes to a cellular subtype of hemangioblastoma. It is sometimes difficult to distinguish hemangioblastoma from a second tumor of Hippel-Lindau disease. With a complete resection of the tumor, however, the prognosis is relatively good.
Prevention
According to the current state of knowledge of medical and pharmacological research, no effective measures to prevent hemangioblastomas are known. Because the causes for the formation of this type of tumors are still largely unexplained.
For this reason, the timely diagnosis and therapy of hemangioblastoma play the most important role. In the case of characteristic complaints and symptoms, a suitable specialist should be consulted as soon as possible.
Aftercare
Cancer treatment is always followed by follow-up care. Because there is a risk that a new tumor will develop in the same place. Physicians perform at least quarterly follow-ups during the first year of diagnosis. Then the rhythm increases. If there is still no new growth in the fifth year, a one-year check is then due.
The patient receives detailed information on this. Follow-up care often takes place in the clinic of the first intervention. Imaging methods such as MRI and CT are used to diagnose hemangioblastoma. In rare cases, the disease requires long-term follow-up care because consequential damage persists. These can be treated with various therapies.
A rehabilitation measure promises quick success. Experts from various fields are available here and can employ the patient in a targeted manner for everyday life. Appropriate medication can also be prescribed in this way. Neurological problems sometimes require fundamental changes in life.
This can cause psychological distress. Psychotherapy can then help. However, it should be noted that a hemangioblastoma is a benign tumor. Consequential damage that affects everyday life is an exception.
You can do that yourself
Those affected with a hemangioblastoma have no options for self-help. This tumor must always be treated by a doctor, which usually requires surgery or radiation.
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 loads. Bed rest and relaxation techniques can have a positive effect on the disease. Patients also 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 conversations. Children should also be fully informed about the possible course of this disease. In many cases, talking to other people affected or, in the case of severe mental stress, talking to a therapist can 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 sign. Regular examinations are also necessary after the treatment in order to detect and treat possible further tumors at an early stage.
