Gliomatosis cerebri
Classification according to ICD-10 | |
---|---|
C71.8 | Malignant neoplasm of the brain, overlapping several areas |
ICD-10 online (WHO version 2019) |
As Gliomatosis cerebri , a primary is brain tumor called in which the diffuse infiltration of the brain tissue, the other gliomas features, as far as is in the foreground that solid proportions not occur. Overall, the disease is very rare, but it can be assumed that misdiagnoses are frequent due to the fact that it is not well known. Some forms of encephalitis may be clinically similar.
Epidemiology
Only about 200 cases have been described worldwide so far. Adults are usually affected, but individual cases have also been observed in children.
Cases in dogs have now also been described.
histology
The original tissue is glial , whereby the distinction between astrocytic and oligodendrocytic cell lines cannot necessarily be made. The involvement of both cerebral hemispheres is typical ; the brain stem , cerebellum and spinal cord can also be affected. The exact mechanisms of formation are unclear, a progressive dedifferentiation is assumed. The individual cells are in themselves not very malignant, but because of the special growth pattern, gliomatosis cerebri must be counted among the malignant brain tumors. According to the WHO classification of tumors of the central nervous system , it is counted as grade III tumors.
Symptoms
As with other brain tumors, the localization is decisive for the symptoms; headache , seizures and psychological changes are common.
diagnosis
The diagnosis is made through imaging ( computed tomography , MRI ) and / or biopsy .
Hemimegalencephaly is to be distinguished .
therapy
Cerebral gliomatosis is not amenable to surgical treatment. Irradiation is possible, in which case the entire brain and spinal cord often have to be irradiated because of the wide and difficult-to-limit extent of the tumor. For common chemotherapy regimens (e.g. PCV), temporary remissions have been described, temozolomide seems to have a beneficial effect, particularly in slowly progressive gliomatoses. A final cure is not possible. The median survival time is given as 14.5 months.
literature
- K. Poeck, W. Hacke: Neurology. 10th edition. Springer, Berlin 1998, ISBN 3-540-63028-7 .
Web links
Individual evidence
- ^ A. Gruber et al .: Gliomatosis cerebri in a dog. In: J Vet Med A Physiol Pathol Clin Med. (2006); 53 (8), pp. 435-438. PMID 16970635 .
- ↑ M. Sanson et al .: Gliomatosis cerebri. In: Rev Neurol (Paris). (2005); 161 (2), pp. 173-181. PMID 15798516 .