CNS tumours Flashcards

1
Q

Most common brain tumour

A

Is secondaries or metastasis
(commonly from small cell carcinoma of lung)
Most common 1° CNS tumour- glioma (or meningioma)

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2
Q

Most common brain tumour in children

A

Pilocytic astrocytoma

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3
Q

Most common malignancy of brain in children

A

Medulloblastoma

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4
Q

Classification of CNS tumours

A
1. Glial tumours:
 •Astrocytoma
 •Oligodendroglioma
 •Ependymoma
2. Undifferentiated tumours:
 •Medulloblastoma 
3. Meningial tumour:
 •Meningioma
4. Others:
 •Lymphoma
 •Germ cell tumour
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5
Q

Anne Mayo Grading system of CNS tumours

A
A. Atypia 
M. Mitosis
E. Endothelial proliferation
N. Necrosis
4 grades based on these factors:
1. None
2. Atypia
3. Atypia and mitosis
4. All 4
Similar to WHO system
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6
Q

Astrocytoma

4 Classes

A
4 classes:
1. Pilocytic 
2. Diffused fibrillary 
3. Anaplastic 
4. GBM glioblastoma multiformi
Prognosis worsens as we move down the classes
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7
Q

Pilocytic astrocytoma

A

In children
Benign
Most common 1° benign tumour of children
Seen in cerebellum, floor of 4th ventricle

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8
Q

Tumours showing mural nodules

A
  1. Pilocytic astrocytoma

2. Pleomorphic xanthoastrocytoma

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9
Q

Features of pilocytic astrocytoma

A
Gross:
1. Cysts
2. Mural nodules
Histo:
1. Microcysts
2. Rosenthal fibres
3. Eosinophilic granular bodies (EGB)
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10
Q

Glioblastoma multiformi

A

WHO grade 4 type of astrocytoma
Poor prognosis
In elderly
Butterfly tumour (crosses the midline)

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11
Q

Features of glioblastoma multiformi

A

Microscopy:
1. Nuclear pleomorphism
2. High cellularity
3. High mitosis
Endothelial vascular proliferation with glomeruloid bodies
Serpentine geographical necrosis surrounded by pseudopalisading tumour cells

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12
Q

Schiller Duval bodies

A

Glomeruloid bodies are also seen (in addition to glomeruloblastoma multiformi) in yolk sac tumours and are called Schiller Duval bodies

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13
Q

IDH wild type of glioblastoma

A
•Primary glioblastoma consisting 90% of cases
•Supratentorial
•Age of presentation: 55 years
•Extensive necrosis and poor necrosis
Mutations:
1. TERT
2. EGFR
3. PTEN
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14
Q

IDH mutant type of glioblastoma

A
•Secondary glioblastoma consisting 10% of cases
• Infratentorial
• Age of presentation: 45 years
• Mild necrosis and better prognosis
Mutations: p53
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15
Q

Oligodendroglioma

A

WHO grade II
Age: middle-elderly
Usually affects cerebral cortex
Genetics:
1. 90% cases are due to mutations of IDH1 and IDH2 genes
2. Loss of 1p and 19q ➡️ highly chemosensitive

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16
Q

Oligodendroglioma microscopic features

A
  1. Cells with perinuclear halo: fried egg appearance
  2. Calcifications
  3. Anastomosing vascular channels: chicken wire blood vessels
  4. Movement of tumour cells around nerve fingers: perineuronal satellitosis
17
Q

Fried egg appearance is seen in

A
  1. Hairy cell leukaemia

2. Oligodendroglioma

18
Q

Calcifications are shown by following tumours

A

.
C. Craniopharyngioma
O. Oligodendroglioma
M. Meningioma

19
Q

Ependymoma

A
From ependymal lining
Associated with NF-2 gene mutations
Most common site in adults: spinal cord
CSF dissemination is common
Microscopy- perivascular pseudorosettes:
 Tumour cells surrounding a blood vessel
20
Q

Medulloblastoma

A

Undifferentiated tumour ➡️ poor prognosis
Most common 1° malignant brain tumour in children
Drops in CSF ➡️ ‘drop metastasis’
Usually occurs in children in the posterior fossa/ cerebellum
Highly radiosensitive

21
Q

Microscopy of medulloblastoma

A

Sheets of small round blue cells with scanty cytoplasm

Homer Wright rosettes

22
Q

Meningioma

A

Benign tumour of adults
Usually due to NF-2 gene mutations
Progesterone responsive ➡️ increased in pregnancy
Previous radiation exposure is a risk factor

23
Q

Microscopy of meningioma

A
5 types:
F. Fibroblastic
P. Psammomatous
S. Spindle cell
S. Secretory
T. Transitional
24
Q

Psammoma bodies

A
Foci of dystrophic calcifications seen in:
M. Meningioma
P. Papillary carcinoma of thyroid
P. Papillary renal cell carcinoma
P. Prolactinoma
S. Serous cystadenocarcinoma of ovary
25
Q

Types of meningioma

A
1. Atypical meningioma:
 Clear cell, or 
 Choroid 
2. Anaplastic meningioma
 Rhabdoid or
 Papillary
 >20 mitosis/HPF
26
Q

Atypical meningioma characteristics

A
4 or more mitosis/10 HPF
 Or 
At least 3 of the following
1. Increased cellularity
2. Prominent nucleoli
3. High nucleocytoplasmic ratio
4. Necrosis
5. Patternless growth
27
Q

Schwanomma

A

Usually due to NF-2 gene missions on chromosome 22 (merlin)
Arises from inferior vestibulocochlear nerve (VIII)
Well circumscribed encapsulated tumour

28
Q

Microscopy of Schwanomma

A

Microscopy:

  1. Antoni A - hypercellular area
  2. Antoni B - hypocellular area
  3. Verocay bodies - empty spaces in between the above 2
29
Q

Neurofibromas

A

Benign tumour
Mutations in NF-1 gene on chromosome 17 (neurofibromin)
Non encapsulated tumour

30
Q

GFAP (glial fibrillary acidic proteins) positive brain tumours

A
Astrocytoma
Oligodendroglioma 
Ependymoma
Medulloblastoma
Choroid plexus tumours
31
Q

Tuberous sclerosis

A
Autosomal dominant
Caused by mutations in:
 TSC 1 - Hamartin
 TSC 2 - Tuberin
Clinically:
1. Seizures
2. Mental retardation
3. Adenoma sebaceum (angiofibroma of nose)
Increased risk of developing other tumours
32
Q

Tuberous sclerosis increases risk of developing tumours like

A
  1. Heart - Rhabdomyomas
  2. CNS:
    • Subependymal nodules
    • SEGA (subependymal giant cell astrocytoma)
    • Cortical tubers
  3. Kidney - Renal angiomyolipoma
  4. Skin:
    • Ash Leaf macules
    • Shagreen’s patch
  5. Lungs - Lymphangioleiomyomatosis
33
Q

Von Hippel Lindau disease (VHL)

A
Autosomal dominant
Gene on chromosome 3p
In kidney- clear cell renal carcinoma
CNS- cerebellar hemangioblastoma
Pancreas- cysts
Adrenals- pheochromocytoma
Skin- epidermal cysts