primary tumour of cns in adults

1. PRIMARY TUMOUR OF
CNS IN ADULT
DR.PRAMOD MEENA
SR NEUROLOGY
GMC,KOTA

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.

4. 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%)

5. 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%

6. 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%

7. 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.

8. 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.

9. 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.

10. 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 gradeIꓦ- mitotically active, necrosis,rapid pre and post surgical
progression.

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

12. 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%).

13. 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

14. 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



15.  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:
1. Dermoid cyst
2. Epidermoid cyst
3. Arachnoid cyst

16.  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

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

39. 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)

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

43. 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.

47. 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.

49. • Methotrexate- based chemotherapy given in high doses (HD MTX
,above 3.5g/m2 )
• 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.

53. 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.

54. REFERENCES
• Bradely′s Neurology in clinical practice, 8th edition.
• Osborn′s Diagnostic Brain Imaging, 3rd edition.
• Louis DN, Ohgaki H, Wiestler OD CW.(2016) WHO classification of Tumours
of the central nervous system( revised 4th 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.
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