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15. Central Nervous System Pathology > 5. Neoplasms > Flashcards

5. Neoplasms Flashcards

1
Q

Types of CNS neoplasms

A
  1. Meningiomas
  2. Gliomas
    - Astrocytoma
    - Oligodendroma
    - Ependymoma
  3. Tumours of neurons
    - Central neurocytoma
    - Neuroblastoma (primitive neuroectodermal tumour)
    - Medulloblastoma
  4. Primary CNS lymphomas
  5. Sellar tumours
    - Pituitary adenoma
    - Craniopharyngioma
  6. Pineal tumours
    - Pineocytoma
    - Pineoblastoma
    - Germ cell tumours of CNS
  7. Peripheral nerve sheath tumours
    - Schwannoma (e.g Acoustic neuroma)
    - Neurofibroma
    - Malignant peripheral nerve sheath tumours
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2
Q

Neoplasms differentials by age

A
  1. Children
    - Primary tumours > secondary tumours (3:1)
  2. Adults
    - Secondary tumours > primary tumours
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3
Q

Differentials by clinical history

A
  1. Known primary malignancy outside CNS
    - Consider secondary metastatic cancer to CNS
    - Usually multiple mets, but not always
  2. Immunosuppression (HIV, chemotherapy)
    - Consider CNS lymphoma or CNS infection mimicking tumour
  3. Familial syndromes
    - Neurofibromatosis-1
    - Neurofibromatosis-2
    - Tuberous sclerosis
    - Von Hippel-Lindau syndrome
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4
Q

Differentials by sites

A
  1. Meninges
    - Meningioma
  2. Intra-/periventricular
    - Ependymoma#
    - Choroid plexus tumour#
    - Neurocytoma (specifically 3rd ventricle)
  3. Midline/sellar region
    - Pituitary adenoma
    - Cranipharyngioma#
    - Germ cell tumours#
  4. Supratentorial
    - Neuroblastoma#
    - CNS lymphoma
    - Gliomas (pilocytic astrocytoma#, glioblastoma multiforme, oligodendroma)
  5. Infratentorial
    - Medulloblastoma#
    - Gliomas (pilocytic astrocytoma#, glioblastoma multiforme, oligodendroma)

= tumours seen in children

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5
Q

Features of meningioma

A
  1. Originates from meningothelial cells (cerebral convexities, falx cerebri)
  2. Location: adherent to meninges
  3. WHO grade I-III (type I most common)
  4. Common & slow-growing, may invade skull bone
  5. Females > males
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6
Q

Molecular pathogenesis of meningioma

A

Loss of 22q (NF-2 gene) - hence seen as one of the

tumours in neurofibromatosis-2

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7
Q

Morphology of meningioma

A
  1. Grossly: tumour stuck onto meninges

2. Histologically: psammoma bodies

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8
Q

Types of Gilomas

A
  1. Astrocytoma
  2. Oligodendroma
  3. Ependymoma
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9
Q

Features of astrocytoma

A
  1. Originates from astrocytes
  2. Location: supra-/infratentorial
  3. WHO grade I-IV
  4. Grade I: Pilocytic astrocytoma (seen in children)
  5. Grade IV: Glioblastoma multiforme (seen in middle-
    aged & elderly, very bad prognosis)
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10
Q

Morphology of astrocytoma

A
  1. [Pilocytic astrocytoma] may have cystic component
  2. [Glioblastoma multiforme] ‘butterfly tumour’, very aggressive, crosses midline, palisading necrosis, nuclear pleomorphism
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11
Q

Features of oligodendroma

A
  1. Originates from oligodendrocytes (constitutes 5-25% of all gliomas)
  2. Location: cerebral cortex
  3. WHO grade I-III
  4. Headaches & seizures
  5. Responds to chemotherapy
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12
Q

Molecular pathogenesis of oligodendroma

A
  1. Oligodendrocytes → Oligodendroma WHO grade I:
    LOH 1p, LOH19q, LOH 4q
  2. Oligodendroma
    - WHO grade I → Anaplastic oligodendroma WHO grade III: CDKN2A deletion, CDKN2C mutation/deletion, LOH 9p & 10q
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13
Q

Morphology of oligodendroma

A

Histologically: fried-egg appearance

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14
Q

Features of epdenymoma

A
  1. Originates from ependymal cells lining ventricular system
  2. Location: intra-/periventricular
  3. Can cause hydrocephalus
  4. Seen in children
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15
Q

Molecular Pathogenesis of ependymoma

A

Some relation to neurofibromatosis-2

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16
Q

Morphology of ependymoma

A

Histologically: rosettes

17
Q

Related tumour to ependymoma

A

Choroid plexus tumour

18
Q

Choroid plexus tumour

A
  1. Papilloma (in children) or carcinoma

2. Can cause hydrocephalus

19
Q

Classification of Tumours of Neurons

A
  1. Mature: central neurocytomas

2. Immature: neuroblastoma, medulloblastoma

20
Q

Features of central neurocytoma

A
  1. Originates from neurons
  2. Location: near 3rd ventricle
  3. Can cause raised intracranial pressure
  4. Relatively good prognosis
  5. Seen in adults
21
Q

Features of neuroblastoma (primitive neuroectodermal tumour)

A
  1. Originates from neurons, supratentorial counterpart of medulloblastoma
  2. Location: supratentorial
  3. Bad prognosis
  4. Seen in children
22
Q

Molecular Pathogenesis of neuroblastoma

A

Differs from that of medulloblastoma even though it

is said to be the supratentorial counterpart of medulloblastoma

23
Q

Features of medulloblastoma

A
  1. Originates from neurons, infratentorial counterpart of neuroblastoma
  2. Location: infratentorial (cerebellum, brainstem)
  3. Aggressive, spreads via CSF
  4. Treatment: surgery & radiotherapy
  5. Seen in children (accounts for 20% of brain tumours
    in children)
24
Q

Molecular pathogenesis of medulloblastoma

A
  1. Loss of 17p
  2. Alterations in Sonic hedgehog/patched pathway
  3. Alterations in Wnt signaling pathway
25
Q

Morphology of medulloblastoma

A

Histologically: may form rosettes (but looks different

from those in ependymomas)

26
Q

Features of primary CNS lymphoma

A
  1. Mostly non-Hodgkin B-cell lymphomas
  2. Location: supratentorial (multiple lesions)
  3. Associations: immunosuppression (HIV), EBV
27
Q

Types of sellar tumours

A
  1. Pituitary adenoma

2. Craniopharyngioma

28
Q

Features of pituitary adenoma

A
  1. Originates from pituitary gland
  2. Location: midline/sellar region
  3. May result in optic chiasma compression (bitemporal
    hemianopia) & endocrine effects (overproduction of pituitary hormone, pressure atrophy of normal pituitary tissue)
29
Q

Features of craniopharyngioma

A
  1. Originates from tooth-forming epithelium within the suprasellar region (embryonal remnant)
  2. Location: midline/sellar region
  3. May cause similar clinical manifestations as pituitary
    adenoma
  4. Good prognosis, but may recur
  5. Seen in children & adults (50-60 years old)
30
Q

Morphology of craniopharyngioma

A
  1. Grossly: cystic, containing yellow viscous engine oil-like fluid
  2. Histologically: Cysts lined by stratified squamous
    epithelium
31
Q

Types of pineal tumours

A
  1. Pineocytoma
  2. Pineoblastoma (resembles neuroblastoma)
  3. Germ cell tumours of CNS
32
Q

Features of germ cell tumours of CNS

A
  1. Takes several forms: teratomas (cystic, may be benign or malignant), germinomas, embryonal carcinomas, endodermal sinus tumours, choriocarcinomas
  2. Location: midline (pineal gland > suprasellar region)
  3. Males > females
  4. Seen more in children than adults
33
Q

Types of peripheral nerve sheath tumours

A
  1. Schwannoma (e.g Acoustic neuroma)
  2. Neurofibroma
  3. Malignant peripheral nerve sheath tumours
34
Q

Features of schwannoma

A
  1. Originates from Schwann cell

2. Location: cerebello-pontine angle

35
Q

Features of malignant peripheral nerve sheath tumours

A

Seen in neurofibromatosis

15. Central Nervous System Pathology (8 decks)

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