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

Histological findings of cerebral gliomatosis

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

Diffuse infiltration of the brain parenchyma by the gliomatosis in a magnetic resonance imaging of the brain (T2-FLAIR weighting). The flat bright (hyperintense) areas correspond to damaged brain tissue.

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

Commons : Gliomatosis cerebri  - album with pictures, videos and audio files

Individual evidence

1. ^ 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 .
2. ↑ M. Sanson et al .: Gliomatosis cerebri. In: Rev Neurol (Paris). (2005); 161 (2), pp. 173-181. PMID 15798516 .

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