Gliosarcoma
| Classification according to ICD-10 | |
|---|---|
| C71 | Malignant neoplasm of the brain |
| C71.0 | Cerebrum, excluding lobes and ventricles |
| C71.1 | Frontal lobes |
| C71.2 | Temporal lobe |
| C71.3 | Parietal lobes |
| C71.4 | Occipital lobe |
| C71.5 | Cerebral ventricle |
| C71.6 | cerebellum |
| C71.7 | Brain stem |
| C71.8 | Brain, overlapping several areas |
| C71.9 | Brain, unspecified |
| ICD-10 online (WHO version 2019) | |
The gliosarcoma is a rare, highly malignant ( malignant ), the brain's own tumor . Like the glioblastoma, which is similar to it, it shows multiforme similarities with the glial cells ( astrocytes ). As a variant of glioblastoma, gliosarcoma is classified as grade IV in the WHO classification of tumors of the central nervous system because of its very poor prognosis .
Epidemiology
Gliosarcoma is a rare neoplasm; there is one tumor for every 50 glioblastomas. This means that for every million inhabitants there is only one new case every two years ( incidence rate 0.05 / 100,000).
pathology
By definition, it is closely related to glioblastoma, but has a sarcomatous (connective tissue tumor) component in addition to the glial component (which usually has astrocytic differentiation ). The latter usually corresponds to a malignant fibrous histiocytoma or fibrosarcoma . The glial part is usually GFAP- positive, the sarcomatous part does not express GFAP.
genetics
The sarcoma-like tumor components are likely to have arisen from mesenchymal transdifferentiation of individual glioma cells. The molecular genetic changes are similar to glioblastoma (loss of chromosome 10 , mutations in the genes TP53 , PTEN , CDKN2A / p16 and other) Unlike ordinary glioblastoma is the rate of EGFR - amplifications , however, significantly lower. The genetic changes in the gliomatous and sarcomatous components are the same, so that one assumes a monoclonal origin of the gliosarcoma.
clinic
The clinical symptoms as well as diagnostic and therapeutic principles correspond to those of glioblastoma.
forecast
While a first study implied a slightly better course of patients with a gliosarcoma compared to patients with a glioblastoma, the following larger studies could not show any significant prognostic difference between the two tumor entities.
literature
- Peiffer, Schröder, Paulus: Neuropathology: morphological diagnosis of diseases of the nervous system and the skeletal muscles. Springer, Berlin 2002, ISBN 3-540-41333-2 .
- KR Kozak, A. Mahadevan, JS Moody: Adult gliosarcoma: Epidemiology, natural history and factors associated with outcome. In: Neuro-Oncology (2008). PMID 18780813 .
- JM Meis, KL Martz, JS Nelson: Mixed glioblastoma multiforme and sarcoma. A clinicopathologic study of 26 radiation therapy oncology group cases. In: Cancer , (1991); 67 (9), pp. 2342-2349. PMID 1849447 .
- RM Reis, D. Konu-Lebleblicioglu, JM Lopes, P. Kleihues, H. Ohgaki: Genetic profile of the gliosarcoma. In: The American Journal of Pathology . (2000); 156, pp. 425-432.
- B. Actor, JM Cobbers, R. Buschges, M. Wolter, CB Knobbe, P. Lichter, G. Reifenberger, RG Weber: Comprehensive analysis of genomic alterations in gliosarcoma and its two tissue components. In: Genes, Chromosomes & Cancer . (2002); 34, pp. 416-427.
- F. Maiuri, L. Stella, D. Benvenuti, A. Giamundo, G. Pettinato: Cerebral gliosarcomas: correlation of computed tomographic findings, surgical aspect, pathological features, and prognosis. In: Neurosurgery (1990); 26, pp. 261-267.
- JR Perry, LC Ang, JM Bilbao, PJ Muiler: Clinicopathologic features of primary and postirradiation cerebral gliosarcoma. In: Cancer. (1995); 75, pp. 2910-2918.
- E. Galanis, JC Buckner, RP Dinapoli, BW Scheithauer, RB Jenkins, CH Wang, JR O'Fallon, G. Farr: Clinical outcome of gliosarcoma compared with glioblastoma multiforme: North Central Cancer Treatment Group results. In: Journal of Neurosurgery . (1998); 89, pp. 425-430.
