Hirneigene Tumoren

Zusammenfassung

Mit einer Inzidenz von etwa 20/100 000 pro Jahr stellen hirneigene Tumoren eine seltene und in vielen Fällen nicht heilbare Erkrankung dar. Die häufigsten hirneigenen Tumoren bei Erwachsenen neben Meningeomen sind Gliome (30% aller Hirntumoren), mehr als die Hälfte der Gliome sind Glioblastome. Auch vermeintlich niedermaligne Hirntumoren können infiltrierend wachsen, eine kurative Behandlung wird dadurch unmöglich. Nachdem sich das Stammzellkonzept bezüglich Tumorentstehung und -resistenz durchgesetzt hat, konnte gezeigt werden, dass genetische Veränderungen an der Entstehung und Progression von Hirntumoren beteiligt sind. Von klinischer Relevanz sind drei molekulare Marker: 1p-/19q-Co-Deletion, Methylierung von MGMT und Mutation von IDH-1 und -2. Die Operation bildet neben der Diagnosesicherung den ersten Teil der Therapie, der sich je nach Entität noch eine weitere Stahlen- und/ oder Chemotherapie anschließen kann. In den vergangenen Jahren konnte gezeigt werden, dass das Resektionsausmaß ein signifikanter Faktor für die weitere Prognose darstellt. Die Therapie von Hirntumorpatienten sollte an neurochirurgischen Zentren mit genügend Expertise erfolgen, da der technische Aufwand hoch ist und nicht in allen Kliniken zur Verfügung steht.

Summary

Brain tumors occur with an incidence of 20/100 000 per year. It is a rare and in most of the cases incurable disease. Meningiomas and gliomas represent 30% of all brain tumors. More than the 50% of the gliomas are glioblastomas and low grade gliomas undergo often a transformation to high grade tumors. Recently, the stem cell concept has been accepted as the most likely pathophysiological mechanism in malignant brain tumors with resulting implications for posttreatment glioma recurrence. On the other hand, it has been shown that molecular markers like 1p/19q, mutations in IDH-1 and -2 MGMT are involved in development and progression of brain tumors. Tumor resection or biopsy aiming a diagnostic confirmation is the first step in management and therapy, followed by further individual radio- and/or chemotherapy depending on the entity of the tumor. Recent findings show that extent of resection is a significant factor for the prognosis. Today, treatment and follow-up of primary brain tumors should be performed in specialized neurosurgical centers with sufficient expertise in regard of the personal and technical effort.

Schlüsselwörter

Hirneigene Tumoren - Gliome - Pathogenese - Diagnostik - operative Therapie

Keywords

Braintumor - glioma - pathogenesis - diagnostics - surgical therapy

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