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Cns tumors

1. PRIMARY TUMOUR OF CNS IN ADULT DR.DHIRAJ

2. INTRODUCTION Primary brain tumours are a diverse group of neoplasm arising from different cells of the central nervous system. It accounts for about 2% of all cancers with an overall annual incidence of 22 per 1,00,000 population. Most common brain tumour in adults is Brain Metastasis . Meningiomas are the most common non- maliganant primary brain tumour f/b Pituitary and nerve sheath tumours . Gliomas accounts for 75% of malignant brain tumours , in which more than half are glioblastomas .

3. PRIMARY CNS NEOPLASMS BY LOCATION Meninges (36%) Cerebral hemispheres(31%) Sellar region(17%) Cranial nerves (7%) Brainstem, cerebellum(4%) Spinal cord/ cauda equina (3%) Ventricles(1%) Miscellaneous(1%)

4. BRAIN TUMOUR INCIDENCE BY AGE GROUP CHILDREN( 0-14 YEARS OLD) Pilocytic astrocytoma,18% Neoplasm,15% Malignant glioma ,15% Astrocytoma ,11% Neuronal/ mixed glioneuronal,6% Ependymal ,6% Nerve sheath,5% Pituitary,4% Craniopharyngioma,4%

5. ADOLESCENTS (15-19 YEARS OLD) Pituitary,27% Pilocytic astrocytoma,10% Other astrocytoma,8% Neuronal, mixed glioneuronal,8% Nerve sheath,6% Meningioma,5% Germ cell,4% Ependymal,4% Embronal tumors,4% Glioblastoma,3%

6. ADULTS (20 + YEARS OLD) Metastatic,50% Primary,50% Meningioma,18% Glioblastoma,7% Pituitary, 7% Nerve sheath tumour,4% Other astrocytoma, 3% Lymphoma ,2% Oligodendroglioma , 2% All other, 7% Most brain tumours have male predominance except meningioma and low grade astrocytoma.

7. RISK FACTORS Established Ionizing radiation Genetic predisposition Not established Head trauma Electromagnetic field radiation Radiofrequency and cellular phones N- nitroso compounds Vitamin C and E Allergies/infection association Tea and coffee Occupational Tobacco, alcohol consumption.

8. CLASSIFICATION Brain tumors are classified according to the WHO CNS tumours grading system. Previously, primary CNS tumours were defined on the basis of histological criteria & assigned a grade ( from I to Iꓦ) In 2016, the classification was revised from the 2007 classification to incorporate signature molecular genetic alterations to the classic histology.

9. WHO Gradings :-only for Glioma WHO grade I – low proliferative potential. possible care with surgery alone. WHO grade II- infiltrating but low in mitotic activity. Can recur and progress to other grades. WHO grade III- Histologic evidence of malignancy( mitotic activity), infiltrative,anaplastic . WHO gradeI ꓦ- mitotically active, necrosis,rapid pre and post surgical progression.

10. CLINICAL FEATURES Generalized Headache Nausea and vomiting Syncope Mental status and behavioral changes Seizure Focal Focal motor weakness Ataxia Seizure Aphasia Visual dysfunction

11. HEADACHE 50-70% patients Bifrontal and tension-like, with constant, dull pressure type Classic brain tumour headache occur in the early morning with nausea and vomiting and improve over the course of the day Only occur in 5-17% of all brain tumour patients, 42% of whom have posterior fossa tumour More common in brain metastases and glioblastomas (90%).

12. Diagnostic investigations MRI Brain with Contrast is the investigation of choice. Diffusion-weighted imaging, diffusion tensor imaging, MR perfusion & MR spectroscopy are used to better characterize the tumour cellularity, vascularity and metabolism respectively. Can distinguish tumour , from non neoplastic processes,including treatment effect. Surgical biopsy

13. CT HEAD:- Intra axial tumours - usually low attenuation high attenuation areas within a tumour calcification, hemorrhage and lymphoma Extra axial: bone erosion and hyperostosis MRI Brain:- TIWI: low signal intensity T2WI/ FLAIR: High signal intensity

14. LOW SIGNAL INTENSITY IN T2WI : 1. CNS Lymphoma 2.PNET 3.Metastasis( melanoma) 4.GBM (less common) 5.Meningioma( less common) ENHANCEMENT: Almost all tumors except Low grade glioma (WHO II & III) CYSTIC NON- tumoral lesions: Dermoid cyst Epidermoid cyst Arachnoid cyst

15. Homogeneous enhancement seen in: 1.Metastases 2.Lymphoma 3.Germinoma And Other Pineal Gland Tumours 4.Pituitary Astrocytoma And Hemangioblastoma 5.Ganglioglioma 6.Meningioma and schwannoma Patchy enhancement seen in: 1.Metastases 2.Glioblastoma multiforme 3.Radiation necrosis

16. Ring enhancement :- Metastasis High-grade Glioma Diffusion restriction :- CNS Lymphoma Oligodendroglioma MR Spectroscopy :- Decreased NAA, increased choline ( Ch /NAA ratio ˃1.3)

17. Mixed Neuronal-Glial Tumours - Ganglioglioma Sezure are the most common manifestation. MC location- supratentorial (temporal>frontal) Children and young adults 30-50% calcification Presenting as cyst-mural enhancing nodule Gross total resection results in survival ranging from 7 to 17 years. Adjuvant irradiation for incompletely resected or anaplastic progression (survival of 3years or less)

18. CHOROID PLEXUS TUMOUR : Includes papilloma and carcinoma Tumour of childhood In adults it account for only 0.2% of all intracranial neoplasm. Located in Lateral ventricle(mc) the cerebello-pontine angle fourth ventricles.

19. Meningeal tumours - Meningioma Most common primary intracranial tumours Older adult Incidentally found asymptomatic meningiomas ( lacking mass effect or compression of a venous sinus) When seizure occur , tumour grow or focal signs emerge Surgical can be curative ,especially in meningioma overlying the hemisphere.

20. PRIMARY CENTRAL NERVOUS SYSTEM LYMPHOMA An uncommon variant of Extranodal Non-Hodgkin Lymphoma. Involves the Brain (periventricular), leptomeninges , eyes or spinal cord without evidence of systemic disease. Most cases are diagnosed in patients between 45 and 65 year of age, median age( fifth decade) ( non-HIV related PCNSL) Homogenous enhancement and diffusion restriction. The most notable risk factor is immunodeficiency Highly aggressive tumour . Left untreated, most patients succumb within 6 months.

21. Methotrexate- based chemotherapy given in high doses (HD MTX ,above 3.5g/m 2 ) F/b Leucovorin rescue has been shown to be the single most effective treatment for PCNSL. For PCNSL in AIDS patients, WBRT has been the standard treatment resulting in poor and non durable response.

22. Conclusion Primary brain tumors remain difficult and challenging disease to manage despite substantial progress in understanding their genesis. Treatments and better outcomes for primary brain tumors have long lagged behind those of other tumours . Combinational regimens will be required to achieve a broad and durable antitumor benefit. New advances in cell engineering technologies and infusion of exvivo prepared immune cells are promising strategies. The present challenge is to translate this better understanding of the pathophysiology into effective therapies.

23. REFERENCES Bradely′s Neurology in clinical practice, 8 th edition. Osborn′s Diagnostic Brain Imaging, 3 rd edition. Louis DN, Ohgaki H, Wiestler OD CW.(2016) WHO classification of Tumours of the central nervous system( revised 4 th edition).WHO Lyon ,2016 Ostrom QT , Gittleman H,LiaoP , et al.CBTRUS stastical report. Primary brain and other central nervous system tumours diagnosed in the united states in 2010-2014.neuro Oncol 2017;19:1-8 Weller M, van den Bent M, Tonn jc,et al.European Association for Neuro -oncology(EANO) guideline on the diangnosis and treatment of adult astrocytic and oligodendroglial gliomas.Rev Lancet Oncol 2017;18:315-29. Up TO Date