Gliosarcoma

The Gliosarcoma is a rare, highly malignant ( malignant ) brain tumor owner. He has like him like glioblastoma multiforme similarities with the glial cells ( astrocytes). As a variant of glioblastoma, the gliosarcoma is classified as grade IV because of its very poor prognosis in the WHO classification of tumors of the central nervous system.

Epidemiology

The Gliosarcoma is a rare neoplasm, to about 50 glioblastomas is one of these tumors. This means that one million inhabitants just about every two years a new disease case comes (incidence rate of 0.05 / 100,000 ).

Pathology

By definition, it has a high affinity with glioblastoma, but has in addition to the glial component ( this has mostly astrocytic differentiation on ) a sarcomatous ( connective tissue tumor ) fraction. The latter corresponds in itself usually a malignant fibrous histiocytoma or fibrosarcoma. The glial component is GFAP - positive as a rule, the sarcomatous portion does not express GFAP.

Genetics

Probably the sarcoma -like tumor components are formed by mesenchymal transdifferentiation of individual glioma cells. The molecular genetic changes are similar to glioblastomas ( loss of chromosome 10, mutations in the genes TP53, PTEN, and other CDKN2A/p16 ) In contrast to the ordinary glioblastoma, the rate of EGFR amplification, however, is significantly lower. The genetic changes are gliomatöse and sarcomatous component the same, so that one starts from a monoclonal origin of gliosarcoma.

Clinic

The clinical manifestations and diagnostic and therapeutic principles are similar to those of glioblastoma.

Forecast

While a first study a low-grade better course of patients implied a gliosarcoma compared to patients with glioblastoma, the following major studies could demonstrate no significant prognostic difference between the two tumor entities.