WHO CNS 5 2021 PEDIATRIC TUMOR CLASSIFICATION AND RADIOLOGY OF MOST COMMON PEDIATRIC POSTERIOR FOSSA TUMORS .pptx.pptx

WHO CNS 5 2021 PEDIATRIC TUMOR CLASSIFICATION AND RADIOLOGY OF MOST COMMON PEDIATRIC POSTERIOR FOSSA TUMORS

1. WHO classification of pediatric brain tumors and radiology of common pediatric posterior fossa tumors DR SARIYA SHAIK NS Resident

2. WHO CNS 5 2021 Classification PEDIATRIC TYPE LOW GRADE GLIOMA PEDIATRIC TYPE HIGH GRADE GLIOMA EPENDYMOMAS EMBRYONAL TUMORS MESENCHYMAL TUMORS

3. PEDIATRIC TYPE LOW GRADE GLIOMAS Diffuse Astrocytoma MYB / MYBL1 Angio centric Glioma Diffuse low grade glioma MAPK Polymorphus low grade neuro epithelial tumor of young CHARACTER Diffusely infiltrating , well differentiated neoplastic cells , fusion with a partner gene Thin cytogenic bland of bipolar cells around peri vesicular spaces ( MYB QK1) Mixed morphology ( BRAF mutation and FGFR1 duplication) Round cells with peri nuclear halos and nuclear pleomorphisms WHO Grade Grade 1 Grade 1 Non specific Grade 1 LOCATION Cerebral hemispheres ( T ) Supra tentorial Cerebral hemispheres or PF Supratentorial CLINICAL FEATURES LEATS LEATS and DRS Raised ICP PROGNOSIS 90% seizure free Favorable Better

4. PEDIATRIC TYPEHIGH GRADE GLIOMA Diffus e Midline Glioma H3 K27 Diffuse Hemispheric Glioma H3, G34 Infant Hemispheric Glioma CHARACTER Aggressive with high grade features infiltrating adjacent and distant brain structures Highly aggressive resembling embryonal tumors Infant , High grade features RTK fusion WHO Grade 4 4 LOCATION Brainstem(Pons) , thalamus , spinal cord Temporal / Parietal lobe CLINICAL FEATURES Cranial nerve palsy's, Motor weakness with ataxia , Long tract signs Raised ICP PROGNOSIS Poor with 2 yr survival rate <10 % Poor prognosis with survival rate of 12 – 24 months

5. EPENDYMOMA Supra Tentorial ZFTA Supra Tentorial YAP1 Posterior Fossa A Posterior fossa B CHARACTER ZFTA gene fused with RELA gene causing activation of NF – KappaB1 signaling YAP1 gene fused wit MAMLD1 causing increased oncogenic activity of NF 1 Immuno staining showing l oss of H3K27m3, over expression of EZHIP H3K27m3 + nt (NS) , Chromosomal instability (M6, T18) LOCATION Frontal or parietal lobe Frontal or parietal lobe 4 TH Ventricle or CP angle 4 TH Ventricle or CP angle WHO Grade 2/ 3 2/3 2/3 2/3 PROGNOSIS Worse prognosis Good prognosis Poor prognosis Good prognosis

6. EMBROYONAL TUMORS Medullo blastoma Atypical Teratoid / Rhabdoid Tumor Embryonal tumor with multilayered rossete CNS NB – FOXR2 CNS BCOR – ITD CHARACTER WNT , SHH , Non WNT SHH Highly malignant, Loss of SMARCB1 or SMARCA4 Alteration of C19 MC and DICER1 Grade 4 Grade 4 Location 4 th ventricle or CP angle Cerebral hemisphere , CP angle < 1 yr lethargry , failure to thrive >1 yr eadache and cranial nerve Supra and infra tentorial

7. Medulloblastoma :4 th ventricle roof / dorsal wall

8. Leptomeningeal metastasis

9. Cerebellar hemispheric location

10. PILOCYTIC ASTROCYTOMA

11. HEMANGIOBLASTOMA

12. EPENDYMOMA

13. ATYPICAL TERATOID / RHADOID TUMOR: AT/RT

14. DIFFUSE MIDLINE GLIOMA

15. TECTAL GLIOMA