Cerebellum tumors should be examined under two separate headings as adults and childhood.
Approximately 70-75% of adult brain tumors are located in the brain, while 60-70% of childhood brain tumors are located in the cerebellum. In other words, 2/3 of all adult brain tumors are seen in the brain, while 2/3 of childhood brain tumors are seen in the cerebellum.
While the most common brain tumor in adults is glial tumors (astrocytomas), the most common cerebellum tumor is hemangioblastomas, whose genetic transition is well known.
The most common type of tumor after childhood leukemia is cerebellum tumors. The most common among childhood cerebellum tumors (medulloblastoma, astrocytoma, epandioma, brain stem gliomas, dermoid and epidermoid cysts, etc.).
Among adult cerebellum tumors, the most common tumors (acoustic neuroma, meningioma, arachnoid and epidermoid cysts, plasmacytomas, glomus jugulare tumors, cerebellum metastases) are seen in the ponto-serbellar corner region in adults.
HEMANGIOBLASTOMA(Von-Hippel-Lindau) complex
Although it is the most common primary tumor of the cerebellum, this tumor can also be seen in the brain stem and spinal cord. Although they are seen between 1.5-2.5% of all brain tumors, they constitute 7-12% of cerebellum tumors. Hemangioblastomas are histopathologically benign tumors. Although these tumors are most commonly seen in the cerebellum, they can be seen in any part of the central nervous system (CNS) in the brain stem, spinal cord and rarely in the brain. This disease has been given different names. The definition of hemangioblastoma has been used since the time of Harvey Kusing. Later, it was given different names such as hemangioma, capillary hemangio-endothelioma, Lindau cyst, or Von-Hippel Lindau. Von-Hippel Lindau (VHL) disease is the name given to this complex if more than one hemangioblastoma lesions are found in the brain or cerebellum, and lesions are found in the eye and visceral organs.
History: A young Swedish pathologist (Arvid Lindau) reported that this disease had a component in the cerebellum, eye, and visceral organs in the book he wrote in 1926.
Genetics: While hemangioblastomas in the cerebellum are seen sporadically, those with VHL complex are familial. It is genetically inherited as an autosomal dominant, with equal frequency in both sexes. A more diffuse disorder such as von Reclinghousen’s disease 8 nerve neurofibramolar is a distinct clinical condition.
Clinical complaints and findings;
Although patients with VHL complex can be seen at any age, including the ages of 3 to 83, it is most commonly seen at the age of 30. Headache is the most common and leading complaint. As a general rule, complaints related to increased intracranial pressure (CIBA) are evident in lesions of the cerebellum. Headache, neck stiffness, intermittent complaints similar to electric shock radiating from the neck to the shoulders are seen. The complaint of vomiting may be due to either obstructive hydrocephalus or irritation of vagal nerve nuclei. Dizziness, imbalance complaints are seen after direct pressure on the vestibular nuclei or brain stem. Gait disturbance (ataxia) is common. Papilledema is seen in 80% of cases. Double vision usually occurs due to CIBAS. Although wide-base gait or trunk ataxia is usually seen in midline lesions, dysmetria and intensional tremor are more common in cerebellum hemisphere tumors. Vertical or rotatory nystagmus is mostly seen in brain stem involvement.
Diagnosis;
As in all cerebellar tumors, medicated brain magnetig resonance (MR) technique, one of the brain imaging methods, is a very important, reliable and harmless test method for the recognition of these lesions. Apart from this, the medicated brain computed tomography (CT) technique provides additional information compared to the MR technique in showing whether the lesion has calcification in some tumors such as cerebellum tumors (eg ependymoma). If the doctor thinks that the lesion is hemangioblastoma in the pre-operative examinations, cerebrovascular angiography should be performed and information about the vessels should be obtained. In addition, individuals with suspected VHL disease should be examined with eye and gastrointestinal system examination, and other members of the family with genetic tests.
Treatment;
As with all cerebellum tumors, if the surgeon thinks that the lesion in the cerebellum is hemangioblastoma in the preoperative period, if the patient has enough time (herniation risk), it is important to examine such individuals in terms of entire spinal cord spread, since these lesions spread through the CSF throughout the spinal cord. In the treatment strategy of these lesions, first of all, gross total resection of the lesion should be performed surgically, if possible. Since hemangioblastomas are tumors resistant to radiotherapy, surgical removal of this lesion should be aimed, if possible. Since these tumors are very dense in terms of vascular nutrition, cerebrovascular angiography should be performed first in surgical planning. If these vascular feeders are suitable for closure, it should be known in consultation with interventional radiology before surgery. If these nutrients are snatched during the pre-operative preparation, excessive bleeding does not occur during the operation. In the surgery of these lesions, it should be planned to remove the entire target lesion. If the lesion cannot be removed completely, recurrences are inevitable.
In conclusion
Hemangioblastomas are benign lesions that are usually seen in the cerebellum and at any age. Headache, vomiting and balance disorders are the leading complaints, and then coordination findings due to cerebellum suppression and CIBA signs due to hydrocephalus appear. Brain MRI is the gold standard for diagnosis and cerebral vascular angiography test is required for vascular relationship. Treatment is surgical resection. There is no need for additional oncological treatment in those who have been successfully removed by surgery. The results are excellent.
