neuro-oncology

Question 1

What are the risk factors of neuro-oncology?

Answer 1

• ionising radiation
• hereditary mutations e.g. li-fraumeni syndrome, NF type 1

Question 2

What is the clinical picture of neuro-oncology?

Answer 2

• focal neurological deficits e.g. hemiparesis, hemiplegia
• gait disturbances if cerebellar involvement
• headaches
• clinical signs of increased ICP e.g. dilated pupils, bradycardia, vomiting
• possible seizures or personality changes (Depends on location)
• MRI or CT is a common diagnostic tool

Question 2

What is a primary brain tumour?

Answer 2

• Primary brain tumors arise within the CNS - Are classified based on the growth characteristics and cell type from which the tumor arises

Question 3

What is the growth characteristic classification of PBT?

Answer 3

Low grade : WHO grade I-II
Borders: well defined
Spreading potential: low
Recurrence: low

High grade: WHO grade III-IV
Borders: poorly defined
Spreading potential: high
Recurrence: high

Question 4

What is the cell types PBT may arise from?

Answer 4

• glioma e.g. astrocytoma
• meningioma
• embryonal neuroectodermal e.g. medulloblastoma
• oligodendroglioma

Question 5

What are the genetic characteristics of brain tumours?

Answer 5

• IDH mutation
• MGMT promotor methylation
• 1p/19q co-deletion
• TP53

Question 6

What are the low grade gliomas?

Answer 6

• polycytic astrocytoma (grade 1)
• diffuse astrocytoma (grade 2)

Low grade gliomas mostly carries
IDH mutation or 1p/19q co-deletion
which carries better outcome

Question 7

What are the high grade gliomas?

Answer 7

• Anaplastic astrocytoma (grade 3)
• Glioblastoma multiforme (grade 4)

Question 8

What is a glioblastoma multiforme? mention also gliosarcoma

Answer 8

• GBM is a WHO grade 4
• most malignant CNS tumour with rapid growth
• affects elderly patients
• median survival 15 months

*gliosarcoma most highest degree of malignancy and aggressiveness in high grade gliomas with the worst prognosis

Question 9

What is a polycytic astrocytoma?

Answer 9

• PA is WHO grade 1
• mostly in younger children < 20
• often associated with NF type 1
• slow growing a localised tumour
• found in cerebellum
• favourable long term survival

Question 10

What mode of surgery is used for polycytic astrocytoma?

Answer 10

• fluorescein 5-ALA guided neurosurgery resection

Question 11

ESMO guideline for high grade gliomas

Answer 11

Grade 3: anaplastic astrocytoma

Grade 4: Glioblastoma Multiforme

• Achieve maximal debulking resection

*assess molecular markers e.g. IDH mutation or 1p/19q co-deletion or MGMT methylation/non-methylation

• treat with temozolomide and RT for GBM
• RT or chemo for anaplastic astrocytoma

Question 12

What is oligodendroglioma?

Answer 12

-low grade arising from oligodendrocytes
- anaplastic with IDH mutation 1p/19q mutation
- classic “fried egg” sign on biopsy
- Treated with resection and RT and chemo

Question 13

What is an ependymoma?

Answer 13

• arises from ependymal cells
• affect younger population
• 4th ventricle is most common location in children
• biopsy shows classica “pseudorosettes”
• mostly supratentorial
• Tx with resection and RT
• usually poor prognosis

Question 14

What is a medullablastoma?

Answer 14

• highly malignant tumour, primitive
  neuroectodermal tissue
• most common malignant pediatric tumour
• mostly in infratentorial, cerebellum, 4th ventricle
• Dropping metastasis along cranio-spinal axis
• Tx is resection, RT and chemo
• Tumour is highly radio and chemo sensitive

Question 15

What is an acoustic neuroma (Schwannoma)?

Answer 15

• benign tumour of CN VIII (vestibulocochlear)
• unilateral tinnitus, hypoacusia, vertigo and headache
• if tumour is bilateral = strong association with NF type 2
• Tx: resection
• Prognosis is good if fully resectable

DG: ORL exam, audiometry (sensorine-
ural deafness), *AEP, MR/MSCT of endo-
cranium-pyramide of temporal bone

Large Tumour = poor due to the difficult
to perform radical excision of the tumor
when recurrences are frequent.

Unresectable tumors
lead to death

Question 16

What is a neuroblastoma?

Answer 16

• malignant neuroendocrine tumour in children
• arises from undifferentiated neural crest cells often on adrenal glands (or abdomen, CNS)
• highly aggressive
• younger px = possible auto-SCT
• poor outcomes in elders
• ifosfamide, cisplatin and etopozoide (ICE) in elderly

Question 17

What is a primary CNS lymphoma?

Answer 17

• primary CNS lymphoma affects the brain, meninges, spinal cord, eye
• without detection outside of these structures
• 90% of these are diffuse large B cell lymphomas
• very aggressive
• median age of 60 yrs

CNS DLBCL is of non GCB –post germinal origin (CD20+, bcl6+, MUM1+) which gives poor outcome

Question 18

What are the therapeutic options for primary CNS lymphomas?

Answer 18

< 70 and fit:
- Induction chemo HD MTX
- addition of cytarabine
- addition of Rituximab
- after induction -> RT
- autologous SC Tx is an alternative to RT for younger patients

Unfit and > 70:
- problematic with MTX
- Temozolomide
- addition of Rituximab
- RT after induction chemo

Question 19

ESMO for metastatic CNS tumours?

Answer 19

• Suspicion of brain metastasis
• perform biopsy followed by post-op MRI/CT
• Diagnosis of Brain Metastasis confirmed?
• Assess prognosis level
• Tx varies from surgery, systemic pharmacotherapy or RT to WBRT to palliative care

Question 20

What are common tumours giving rise to metastases in the brain?

Answer 20

Lung cancer (most common)
Breast cancer
Pancreatic cancer