Yüksek gradeli glial tümörler Dr.Gönül Aydın Prof. Dr. Aşkın Görgülü

Yüksek gradeli glial tümörler Gliomlar genel olarak kötü huyludurlar. Kromozom anomalileri, Supressor gen inaktivasyonu, Bazı özel onkogenler(P53) bu tür tümörlerin ortaya çıkmasından sorumlu tutulmaktadırlar.

WHO-2000 Astrositom (WHO grade II) Diffüz, Fibriler, Protoplazmik, Gemistositik Anaplastik astrositom (WHO grade III) Glioblastoma multiforme (WHO grade IV) Pilositik astrositom [WHO grade I] Subependimal dev hücreli astrositom [WHO grade I] Pleomorfik ksantoastrositom [WHO grade I]

Klinik Baş ağrısı (en sık % 54) Motor kuvvetsizlik KİBAS bulguları Nöbet Kranial sinir tutulumu (1-6. kranial sinir) İnfratentoryal tutulumda hidrosefaliye bağlı bulgular

Tanı BT MR Anjiografi

AA-GBM: KONTRAST TUTMA KALIBI HİÇ TUTMAYABİLİR HOMOJEN diffüz/fokal HETEROJEN Neovascularity in GBM. Panel A Left vertebral angiogram shows a small tangle of abnormal arterial vessels (long arrows) and early draining vein (short arrow). This angiographic appearance could also be seen in a small arteriovenous malformation. Panel B Sagittal gadolinium-enhanced T1-weighted image shows an irregular, thick-walled enhancing mass with central nonenhancing regions corresponding to necrosis.

Tedavi Stereotaktik biopsi İnternal dekompresyon Lobektomi Radikal rezeksiyon RT KT  En iyi sonuç; Maksimal güvenli rezeksiyon+RT

İyi prognoz kriterleri Genç yaş Düşük histolojik grade Klinik düzey (Yüksek karnofsky skoru) Cerrahi kalite (Total eksizyon)

Yayılım 1-Beyaz madde içinden ilerleme a-Frontal lobda, korpus kallozumu çaprazlayarak ‘Kelebek gliom’ oluştururlar. b-Serebral pedinkül içinden c-Sentrum semiovale d-Kapsula interna 2-BOS yoluyla (Yüksek gradeli gliomlarda %10-25 meningeal ve ventriküler ekilme kaydedilmiş) 3-Sistemik ( nadir) olarak yayılırlar.

Hemotojen Doğrudan yayılım; uncinate fasikulus Patterns of Dissemination.-Three major patterns of dissemination are seen with GBM. Most frequently, they metastasize from their original location by direct extension, commonly along white matter tracts. One classic example is the spread from a primary lesion in the temporal lobe to the frontal lobe via the uncinate fasciculus (Figure 13). Less commonly, GBM, like other central nervous system neoplasms, may spread via cerebrospinal fluid pathways (Figure 14). Less than 2% of GBMs exhibit cerebrospinal fluid seeding, either within the central nervous system or through ventriculoperitoneal or ventriculopleural shunts. Subependymal spread of GBM is another uncommon but characteristic pattern of dissemination (Figure 14) that correlates with a poor prognosis. Perhaps the least common mode of dissemination is hematogenous spread to extraneural sites. This pattern is so rare that Bailey and Cushing asserted that it did not occur (40). This pathway is a rare cause of dense, osteoblastic bone lesions (Figure 15) and is seen primarily in patients who have undergone surgical treatment of GBM. Dissemination of a primary GBM along white matter tracts. Photograph of a gross pathologic specimen shows a large right frontotemporal GBM that traverses the uncinate fasciculus. Dissemination of a primary GBM via cerebrospinal fluid pathways and subependymal spread. (A and B) Axial Panel A and coronal Panel B gadolinium-enhanced MR images of the same patient demonstrate leptomeningeal seeding by cerebrospinal fluid pathways (arrowheads) and subependymal spread (arrows) of a GBM. Panel C Photograph of an autopsy specimen from a similar case shows diffuse subependymal spread of GBM (arrows). şekli yok: Hematogenous dissemination of GBM. Perhaps the least common mode of dissemination is hematogenous spread to extraneural sites. This pattern is so rare that Bailey and Cushing asserted that it did not occur (40). This pathway is a rare cause of dense, osteoblastic bone lesions (Figure 15) and is seen primarily in patients who have undergone surgical treatment of GBM. Doğrudan yayılım; uncinate fasikulus Karşı hemisfere geçebilir BOS ve subependimal yayılım Hemotojen

Malign Gliomaların İnsidensi (Tüm Yaş Grubu) GBM 50 % Astrositoma 30 %

Anne-Mayo kriterleri; Nükleer atipi Mitoz Endotel proliferasyonu Nekroz

Anne-Mayo grade’ i *Grade *Kriterlerin sayısı 1 2 3 4 3 ya da 4

Anaplastik Astrositom Ortalama yaş 46 Kötü huyludur. Hızlı büyürler. Genellikle Kontrast tutarlar. Frontal ( %40), Temporal (%25), Paryetal (%25) loblarda yerleşim gösterirler. 17p delesyonu, P53 gen inaktivasyonu sıklıkla eşlik eder.

Anaplastik Astrositom Tedavi  Cerrahi eksizyon+RT Rekürrenste  Reoperasyon+KT, RT, İntersisyel brakiterapi Survey 2 yıllık yaşam % 40-50 5 yıllık yaşam % 18

ANAPLASTİK ASTROSİTOMA (GRADE III) Hipersellülarite-sellüler pleomorfizm-atipik nukleuslar-ceşit mitoz

ANAPLASTİK ASTROSİTOMA (GRADE III) T1+C

GBM Ortalama yaş 56 Sıklıkla frontal lobda yerleşim gösterirler. % 80 olguda 10. kromozomda tam veya kısmi kayıplar bildirilmiş. 17p delesyonu, P53 gen inaktivasyonu sıklıkla eşlik eder.

GBM’de Yerleşim Frontal Temporal Paryetal Oksipital %25 %36 %22 % 33

GBM Ortalama yaşam süresi  4 ay Cerrahi+RT  9.25 ay Cerrahi+RT+KT  10 ay 2 yıllık yaşam süresine hastaların % 10 u ulaşabilmiştir.

Typical GBM in the left frontal region Typical GBM in the left frontal region. Axial T2-weighted MR image shows a heterogeneous mass, with focal low signal intensity suggestive of blood products and high signal intensity suggestive of fluid or necrosis. Extensive vasogenic edema surrounds the tumor. Pathology: necrosis and pseudopallisading

Glioblastom multiforme

Glioblastom multiforme

Posterior Fossa GBM Posterior Fossa GBM The most common astrocytoma in the posterior fossa is the juvenile pilocytic astrocytoma, which occurs most often in the cerebellum, hypothalamus, and optic nerve and tracts. Juvenile pilocytic astrocytomas are distinct from diffuse astrocytomas and do not undergo progressive transformation from low-grade to high- grade gliomas. The prevalence of primary GBM of the cerebellum is extremely small, especially compared with the prevalence of this lesion in the supratentorial location. The imaging features of this tumor, when it does occur, are relatively similar to those of GBM in other locations (Figure 22). Differential diagnosis includes metastases, hemangioblastoma, or possibly an atypical medulloblastoma. Astrocytoma of the brain stem is classically seen in children and most commonly is a diffuse, fibrillary tumor of low histologic grade (Figure 23). This tumor does progress to GBM, however, and the tendency for this progression may be slightly increased after radiation therapy. GBM of the brain stem is also seen in the adults. These tumors represent a significant challenge for clinical management. Because even the initial surgical biopsy is associated with risk of injury to vital structures, some clinicians have advocated use of radiation therapy without biopsy in selected cases. GBM of the cerebellum. Coronal ancient gadolinium-enhanced T1-weighted image shows an enhancing mass in the cerebellum, close to the cerebellopontine angle. Differential diagnostic considerations included meningioma and schwannoma, but the lesion proved to be a primary cerebellar GBM at pathologic examination. Photograph of a gross pathologic specimen from a different case demonstrates a cerebellar GBM in the same location. Axial gadolinium-enhanced MR image of a 5-year-old girl shows a pontine GBM.