5. Neoplasms Flashcards

(35 cards)

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
Morphology of medulloblastoma
Histologically: may form rosettes (but looks different | from those in ependymomas)
26
Features of primary CNS lymphoma
1. Mostly non-Hodgkin B-cell lymphomas 2. Location: supratentorial (multiple lesions) 3. Associations: immunosuppression (HIV), EBV
27
Types of sellar tumours
1. Pituitary adenoma | 2. Craniopharyngioma
28
Features of pituitary adenoma
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
Features of craniopharyngioma
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
Morphology of craniopharyngioma
1. Grossly: cystic, containing yellow viscous engine oil-like fluid 2. Histologically: Cysts lined by stratified squamous epithelium
31
Types of pineal tumours
1. Pineocytoma 2. Pineoblastoma (resembles neuroblastoma) 3. Germ cell tumours of CNS
32
Features of germ cell tumours of CNS
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
Types of peripheral nerve sheath tumours
1. Schwannoma (e.g Acoustic neuroma) 2. Neurofibroma 3. Malignant peripheral nerve sheath tumours
34
Features of schwannoma
1. Originates from Schwann cell | 2. Location: cerebello-pontine angle
35
Features of malignant peripheral nerve sheath tumours
Seen in neurofibromatosis