Angioblastoma is the short version for hemangioblastoma. Hemangioblastomas belong to the benign tumors of the central nervous system. They usually grow from the spinal cord or the posterior fossa of the skull.
Angioblastomas can occur sporadically or in a familial cluster and then manifest as Von Hippel-Lindau disease. An angioblastoma typically grows together with a large cyst that contains the actual tumor as a small solid part of the wall. The cyst, which grows along with the tumour, contains an amber-coloured fluid and several of them can appear as so-called syringa in the spinal cord. In angioblastomas there is a strong production of erythropoietin (Epo), which is also used as a doping agent. Due to the increased production of erythropoietin, an increase in red blood cells can occur in the affected patient.
Symptoms
If the hemangioblastoma is located in the cerebellum, the following symptoms are the main ones: If the growth of the benign tumor is more advanced, there may also be disturbances in consciousness. If the tumour is located in the spinal cord, the affected person may show sensory disturbances as well as signs of paralysis. In addition, insecurity of gait and disturbances in bowel movement and urination are possible. However, the tumour rarely causes pain. – Headaches
- Nausea
- Balance disorders
- Gang insecurity
- Rotational vertigo
Therapy
Annual check-ups are necessary in order to keep an eye on the slow but often steadily progressing tumour growth and to be able to intervene in time. Magnetic resonance imaging with contrast medium is the preferred method for primary diagnosis and follow-up. If treatment of the angioblastoma becomes necessary, it is removed by microsurgery. In most cases the hemangioblastomas can be completely removed due to their benignity and usually do not grow back again. Radiation therapy has not yet achieved convincing results in the treatment of angioblastomas and drug treatments of angioblastomas are currently being researched, so that the only proven effective therapy for haemangioblastomas is surgical removal.
Definition
Von Hippel-Lindau’s disease was discovered by the doctors Eugen von Hippel and Arvid Lindau at the beginning of the 20th century as a hereditary tumour syndrome. In addition to haemangioblastomas, the disease also includes retinal tumours (retinal angiomas), renal tumours and adrenal tumours (phaeochromocytomas). Tumours of the inner ear, the epididymis and also of the pancreas can also be included.
During the disease, the patient often develops several hemangioblastomas. Thus, a final cure by surgical removal of the currently existing tumours is usually not possible, as new tumours are always growing back. The removal of angioblastomas from the CNS (central nervous system) is usually successful without long-term damage, but it is still not advisable to remove all tumours from the patient suffering from Hippel-Lindau disease.
Rather, it has proven to be more effective to monitor the patient with von Hippel-Lindau disease annually by means of MRI of the brain and spinal cord. It is then discussed with the patient individually which of the tumours should be removed if possible. Here, priority is given to those tumours that cause symptoms.
If there are tumours that do not cause any symptoms or complaints, but which are growing and becoming larger and larger in the follow-up examinations, removal is also recommended. This is because a study has shown that there is generally no improvement or worsening of the symptoms and functional condition when an angioblastoma is surgically removed. This means that, as a rule, no permanent damage is caused.
On the other hand, it also makes it clear that symptoms that have already arisen cannot be reversed by removing the tumour that caused them. The conclusion can be drawn that it is highly recommended to remove growing tumours as long as they do not cause irreversible symptoms.
