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Neuropysch > CNS tumors > Flashcards

CNS tumors Flashcards

1
Q
  • Childhood CNS tumors occur most often (75%) in the ______
  • Adult CNS tumors are most often_____
  • Localization of adult CNS tumors follows a mass distribution - most occur in the cerebral

hemispheres, most frequently frontal lobes as they are biggest

A

posterior fossa

supratentorial.

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2
Q

General principle of CNS tumors

A
  • Both low and high grade neoplasms have significant morbidity and mortality
  • Diffusely infiltrative
  • Involvement of critical anatomic areas
  • Inability to resect critical anatomic areas
  • Most of the time do not spread outside of brain (metastases: extremely uncommon)

• May spread through subarachnoid space

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3
Q

The most common glial tumor
• Diffuse have an inherent tendency to progress to higher grades (anaplastic astrocytoma, glioblastoma) over time.

Annual incidence ~3-4 per 100,000

At least 80% are glioblastomas

A

Astrocytoma

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4
Q

Clincal features of astrocytomas

A
  • Seizures
  • Focal neurologic deficits – gradual (not abrupt) onset

• Headaches

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5
Q

-cellularity is moderately increased and occasional nuclear atypia

A

Grade 2 – astrocytoma (diffuse)

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6
Q

increased cellularity, distinct nuclear atypia, marked mitotic activity

A

Grade 3 – anaplastic astrocytoma-

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7
Q

pleomorphic astrocytic cells, brisk mitotic activity, prominent microvascular proliferation and/or necrosis and Psuedopalisading cells

A

Grade 4 glioblastoma multiforme –

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8
Q

Gross features of astrocytoma

A

Space occupying lesion; low grade. see expanded and flattened gyri in right frontal lobe

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9
Q
A
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10
Q

What does GBM stain with? What histological features do we see?

A

with GFAP see vascular prliferation; lots of pleomorphic astrocytic cells with brisk mitotic activity

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11
Q
  • Most common glioma in children
  • Typically present in 1st two decades

Found in posterior fossa

A

Pilocytic astrocytoma

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12
Q

Clincal features of pilocytic astrocytoma (common childhood cancer)

A
  • Most commonly occur in cerebellum
  • May also occur in optic nerve, 3rd ventricle, hypothalamus, brainstem, and occasionally cerebral hemispheres
  • Presentation with focal neurologic deficit, seizures, or S/S of increased intracranial pressure
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13
Q

Kid presets with seizures and signs of increased cranial pressure. You see this on imaging. Dx adn prognosis

A

Pilocytic glioblastoma

slow glow, excellent prognosis, surgery = curative

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14
Q
A
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15
Q

Biphasic pattern: densely fibrillary (pilocytic) areas alternating with microcystic component and rosenthal fibers

A

Pilocytic astrocytoma

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16
Q

• Adults in 5th to 6th decade

  • Long history of progressive neurological symptoms (seizures, headache focal signs)
  • Imaging – well defined hypodense/hypointense mass, may see calcification
A

Oligodendroglioma

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17
Q

Prognosis of oligodendroglioma

A

median survival = 5-10 yrs for grade II

better then astrocytomas

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18
Q

Oligodendroglioma: Allelic loss of chromosome ____ and ____ are predictors of prolonged survival and susceptibility to chemotherapy in anaplastic oligodendrogliomas

A

1p and 19q

good to have this shit!

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19
Q

See fried egg appearane on histology with round nuclei

there are calcifications present

A

Oligodendroglioma

(fried eggs are Over easy)

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20
Q

What do we see in grade 3 anaplastic oligodendroglioma

A

vascular porliferation and mitosis is increased; mass lesion that is invasive

(chicken wire is charcteristic)

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21
Q

Typically occurs in children and young adults

Occurs along ventricular system, usually posterior fossa (4th ventricle)

Clinical S/S – _hydrocephalus, occasionally seizures _

A

Endymoma

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22
Q

Grades of ependymomas and prognosis

A
  • Ependymoma (WHO II)
  • Anaplastic ependymoma (WHO III)

Prognosis – average survival for posterior fossa tumors is 4 years

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23
Q

Solid or cystic tumors protruding from ventricular lining & filling ventricle

May invade brain parenchyma May disseminate through subarachnoid space

A

Ependyomama: pt presents with hydrocephalus

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24
Q

See true rossettes (comlnar cells) arranged around a central lumen

A

Ependymoma

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25
Q

Highly cellular tumors with increased mitoses & vascular proliferation Still has perivascular pseudorosettes, and (sometimes) true rosettes

A

Anaplastic ependymoma

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26
Q

Etiology of Gliomas

A

Etiology of gliomas

  • No cause identified in most common types • Familial tumor syndromes (rare: < 5%)
  • Li-Fraumenisyndrome(TP53germlinemutations) • Predominantly astrocytomas
  • Turcotsyndrome(APC&DNAmismatchrepairgenes) • Glioblastoma or medulloblastoma
  • Cowdensyndrome(PTENgenemutation) • Cerebellar gangliocytoma
  • NF1,NF2,Tuberoussclerosis,vonHippel-Lindau(discussedlaterinthissession)
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27
Q

Environmental exposures associated with Gliomas

A

Only environmental factor definitively associated with increased risk of brain tumors is therapeutic radiation

• Example: Children who underwent prophylactic CNS irradiation for acute lymphocytic leukemia have increased risk of development of brain tumors

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28
Q

Typically found in first two decades

Occurs in 4th ventricle, lateral ventricle, 3rd ventricle and cerebello- pontine angle

Presentation with hydrocephalus

  • Overproduction of CSF
  • Obstruction of CSF flow
A

Choroid plexus papilloma

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29
Q

Choroid plexus papilloma vs carcinoma

A

Choroid plexus carcinoma

  • Occurs in children < 10 years
  • Rare in adults
  • Prognosis
  • Choroid plexus papilloma – very good with surgical resection
  • Choroid plexus carcinoma – poor prognosis
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30
Q

Well-demarcated, pedunculated or cauliflower-like mass

Papillomas do not invade adjacent parenchyma, (but carcinomas do)

A

choroid pleuxs papilloma

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31
Q
  • Usually attached to roof of 3rd ventricle
  • Intermittent obstruction of foramen of Munro (often positional)
  • Positional headache
  • Thin-walled cyst lined by cuboid/columnar epithelium
A

Colloid Cyst

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32
Q
  • Usually in first three decades
  • Long standing history of seizures is common
  • Typically supratentorial and in temporal lobe
  • Imaging; solid or cystic, often calcification
  • Surgical resection is usually curative

•No radiation or chemotherapy needed

A

Ganglioma

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33
Q

Typically a well-circumscribed mass, often with a cyst containing a mural nodule

histology: composed of atypical ganglion cells (neurons) and neoplastic glial compoenent

A

Ganglioma

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34
Q

Clincal features associated with medulloblastoma

A

Cerebellar dysfunction (ataxia) and increased intracranial pressure

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35
Q

Characteristic feature: tendency to spread through CSF pathways

A

medulloblastoma

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36
Q

Recently separated into four biologically distinct groups based upon genetic abnormalities

Each has difference risks of recurrence & progression (high, standard, low)

Traditionally a grade IV neoplasm, but now some subgroups have more favorable

outcomes

A

MEdulloblatoma

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37
Q

Treatement of medulloblastoma

A

Treatment – surgical resection followed by radiation (usually cranio- spinal)

• In some subgroups: with total excision and radiation, 5-year survival may be 75%

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38
Q

Solid well-defined homogeneous mass

Tendency to spread through sub- arachnoid space & form “drop” metastases

A

MEdulloblastoma

39
Q

HOmer wright rosettes

synaptophysin immunoreactivity, hihgly cellular and compose of undifferenitated cells

A

Medulloblastoma

40
Q

5-10% of primary intracranial neoplasms

Typically occur between 40-60 years

• Immunocompetent host – 55 yrs (rare, but increasing incidence)

  • Immunocompromised host – 40 yrs • ~10%ofAIDSpatients,usuallylate-stage
  • ~20%ofpost-transplantpatients

• Epstein-Barr virus association

A

Primary CNS lymphoma

41
Q

Boring shit on Primary CNS lymphoma

A

98% B-cell; 2% T-cell

Symptoms are non-specific, referable to mass lesion

2/3 are supratentorial

Meningeal spread in ~25%

Poor prognosis – most die within one year

  • RX – chemotherapy & radiation
  • Spread of disease outside of CNS is rare
42
Q

on MRI see contrast enhancing multiple periventricular lesions

A

pervientricular mass lesions with necrosis seen in Primary CNS lymphoma

43
Q

Perivascular arrangement of neoplastic cells;make a cuff around the vessels

A

PRimary CNS lymphoma

44
Q
  • Most common extra-parenchymal neoplasm of CNS
  • Clinical features: Occurs in middle to late adult life, W>M

Symptoms dependent on location: may lead to increased intracranial pressure, focal signs, seizures

Imaging: dural based, usually well-defined, contrast-enhancing

A

Meningioma

45
Q

Significance of presence of progesterone receptors on cells & increased occurrence in women is not understood

Associations: previous radiotherapy and NF2

Genetic abnormalities: monosomy of chromosome 22 or mutation of NF2 gene (on chr. 22)

A

Meningioma

46
Q

Meningioma:

Significance of presence of______ receptors on cells & increased occurrence in women is not understood

Associations: previous_____ and _____

Genetic abnormalities: monosomy of chromosome____ or mutation of NF2 gene (on chr. 22)

A

progesterone

radiotherapy and NF2

22

47
Q

Location of meningioma

A

Parafalcine, lateral sulcus, orbitofrontal cortex, cerebellopontine, thoracic spinal dura

48
Q

FEature of menigioma:

A

Firm, well-defined, tan-white tumor often attached to dura

49
Q

Typical (WHO grade I): >90% are Grade I
• Excellent 5-year survival
• 20% recur within 10 years (when all was grossly resected)

Atypical (WHO Grade II): 5% are Grade II
• More mitoses, less differentiation, and sometimes brain invasion • 5-year recurrence = 40%, mortality = 20%

Anaplastic (WHO Grade III): 2% are Grade III • Even more mitoses and even less differentiation • median survival = 1.5 years

A

boring shit about menigioma

50
Q

Describe histology of meningioma

A

sheets of tumor cells with indistinc borders: whorls and round to oval nuclie, disperesed chromatin with wispy eosinophilc cytoplasm

51
Q

Meningioma, atypical & anaplastic features

A

Necorsis, mitosis, brain invasion

52
Q

Mets to CNS

A

Metastatic tumors to the CNS

May be first presentation of malignancy (in 50%)

Most originate in the lung or breast carcinomas
• Other common sources are kidney, gastrointestinal tract, and skin (malignant melanoma) neoplasms

53
Q

S/S of mets to CNS

what we see on radiograph

prognosis

A

Clinical S/S

• Headaches, focal neurologic signs, altered mental status

Radiographically – distinct contrast-enhancing mass with surrounding

edema, usually multiple

• Prognosis – poor, most die within a few months

54
Q

PROSTATIC ADENOCARCINOMA

Patients usually present with spinal cord compression

A

Mets to vertebral bodies

55
Q
  • Benign tumor composed of Schwann cells
  • Most common 4th to 6th decades
  • Involve peripheral nerves, usually in head and neck and flexor surfaces of

extremities
• Asymptomatic masses
• Spinal tumors–radicularpain

A

Schwannomas

56
Q

• Intracranial tumors most often in cerebellopontine angle and attached to 8th

nerve
• Symptoms of hearing loss,tinnitis,facial numbness

• Scans show well-defined contrast enhancing mass

A

Schwannomas

57
Q

Schwannomas are associated with

A

NFT2

58
Q

See compact spindle cells or loose spongy areas and small cells with round nuclei

A

Schwannoma

59
Q

General on Neurofibromas (Peripheral nerve sheath tumors)

A

Benign tumor composed of Schwann cells, fibroblasts, and perineural cells

Associated with Neurofibromatosis type 1

Multiple forms

  • Cutaneous (localized) neurofibroma: most common • In dermis or subdermal
  • Usually solitary (not associated with NF1)

• Peripheral nerve : Solitary or Plexiform - usually in NF1

60
Q

On histology see shit that looks like shredded carrots with wavy nuclei, elongated spindle cells and hypocellular; diffusely infiltrative to adj nerve and soft tissue

A

Neurofibromas

61
Q

Occurs almost exclusively in NF1

Transformation of multiple fascicles of nerves into this with preservation of anatomic configuration

Typically affects larger nerves or a plexus

High likelihood of malignant transformation

A

Plexiform neurofibroma

62
Q
  • Mostly in extremities
  • In CNS, assoc with trigeminal nerve

Strong assoc with NF1

High grade, aggressive

Infiltrative,non-encapsulated fleshy masses
• Highlycellular,moderateto marked nuclear pleomorphism

• High mitotic rate

A

Malignant peripheral nerve sheath tumor

63
Q

More Familial Tumor Syndromes

  • Each has cutaneous or eye abnormalities
  • Old name is “Neurophakomatoses”
  • Most are linked to loss of tumor suppressor genes
  • Four types:

(associated with increased risk of nervous system- associated tumors)

A
  • Neurofibromatosis type 1
  • Neurofibromatosis type 2
  • Von Hippel-Lindau disease
  • Tuberous sclerosis
64
Q

• Neurofibromas, café-au-lait spots, Lisch nodules, optic glioma, osseous lesions,

**axillary freckling, family history **

  • Autosomal dominant with Almost complete penetrance
  • However, 50% represent new mutations
  • 1: 3000 individuals (much more common than NF2)
A

Neurofibromatosis 1

(von Recklinghausen disease)

65
Q

Tumors associated with von Recklinghausen

A

• Neurofibromas (all types)

  • Most important is neurofibromas that undergo transformation to malignant peripheral nerve sheath tumors
  • Optic nerve gliomas and other astrocytomas
  • Others (rhabdomyosarcomas, pheochromocytomas, carcinoid tumors)
66
Q

Von Recklinghausen

NF1 gene on ____

• Gene product – ______

A

chr. 17

neurofibromin

67
Q

Function of Neurofibromin seen in von Recklinghause

A

G-protein-dependent signal transduction pathway (at umor suppress or gene) that influences cell proliferation & differentiation

Abundant in Schwann cells and neurons

68
Q

What are some give aways for neurofibromatosis 1

A

axillary freckling

cafe au lait spots

69
Q

Optic nerve gangliomas and Lisch nodules are seen in:

A

This is in Neurofibromatosis or von Recklinghausen

Lische nodules

70
Q

Criteria for NF2

A

Bilateral vestibular schwannomas (most common manifestation),f irst degree relative with NF2, lens opacity, cerebral calcifications

Other associated tumors: meningiomas, schwannomas, gliomas, neurofibromas

(Unlike NF1, plexiform neurofibromas are not found & malignant

transformation of neurofibromas is rare)

71
Q

Genetics of NF2

______ inheritance

gene____ that makes _____

A

Auto D

gene 22

product is Merlin

72
Q

What does ‘merlin’ (gene product of NF2)

A

Tumor suppressor gene which promotes assembly of cell junctions (with loss of gene, cell-to-cell contact is disrupted & this contributes to tumorigenesis)

73
Q

Von Hippel Lindau disease

Pattern:

• Features:

A

Autosomal dominant

• Hemangioblastomas of CNS & retina and Cerebellar hemangioblastomas, renal cell carcinoma, pheocromocytoma, visceral cysts and retinal angiomas

74
Q

VHL gene on chr. 3 is implictaed in what disease, what is it responsible for

A

Von Hippel Lindau disease

• VHL protein controls angiogenesis through regulation of expression of

endothelial growth factor, erythropoietin and other growth factors

75
Q

Hemangioblastoma

Most commonly occurs in______ and Occasionally in cerebrum or spinal cord

Symptoms usually secondary to __________due to obstruction

A

cerebellum

increased intracranial pressure

76
Q

What does hemangioblastoma look like on MRI imaging?

A

Well defined contrst enhancing cystic mass with mural nodule

surgically resectable

77
Q

Numerous vessels interspersed with stromal cells
Stromal cells have abundant foamy cytoplasm (contains fat)

A

Hemangioma!

78
Q

Tuberous sclerosis

genetics:

A
  • Autosomal dominant
  • Positive family history in 50% • Occurs 1 in 6000

TS caused by mutations in 2 tumor suppressor genes

79
Q
  • TSC1 (chr. 9) – codes protein____
  • TSC2 (chr 16) – codes protein______

IMplicated in tuberous slcerosis, what is their role

A

hamartin

tuberin

*** These proteins form dimers & regulate protein synthesis & cell proliferation (inhibit mTOR)

80
Q

Devo of hamartomas and benign neoplasms: cortical hamartomas, subcortical glioneuronal, suependmal giant cell astrocytoma, cutansous angiobriomas, sub ungal fibromas, ect

A

all see in tuberal sclerosis

81
Q

Tuberosclerosis stands for :HAMARTOMAS

A

Harmas in the CNS and skin

Angiofibomas

Mitral regurg

Ash leaf spts

cardiac Rhabdomyoma

Tubero sclerosis

dOminant

Mental retardation

Angiomulomas,

Seizures

Shagreen pathces

82
Q

Neurons haphazardly arranged in cortex

Often have glial as well as neuronal features

A

Tuber histology

83
Q

Most common primary brain tumor

A

meningiomas

followed by gliomas

84
Q

Likely to cause obstructive hydrocephalus.. Exophytic solid well-defined mass arising

in the wall of lateral ventricle

A

Subependymal giant cell astrocytoma

85
Q

Large pleomorphic multinucleated tumor cells with eosinophilic cytoplasm
May be of astrocytic or glioneuronal origin
No malignant transformation, local invasion reported

A

Subependymal giant cell turmo

86
Q

A clinical syndrome produced by remote effect of a systemic malignancy that cannot be attributed to direct invasion by tumor or its metastasis, infection, ischemia, surgery, related metabolic or nutritional disorders or toxic effects of therapy

A

Paraneoplastic Syndromes

87
Q

Most common associated malignancies with paraneoplastic syndromes

A

Most common associated malignancies • Small cell carcinoma
• Gynecologic malignancies

  • Breast, ovary, fallopian tube, peritoneum • Hodgkin’s and non-Hodgkin’s lymphoma
  • Testicular cancer
  • Neuroblastoma
88
Q

Two classes of paraneoplastic syndromes

A
  1. Related to ectopic hormone production: ie. SIADH, ACTH
  2. Neurologic syndromes: rare (0.1% or all cancer pts.; 3% of small cell lung cancer (SCLC) pts)
89
Q

Most common neurologic syndomres, associated with strong FEMALEL predominance in paraneoplastic syndromes

A
  1. Neurologic syndromes: rare (0.1% or all cancer pts.; 3% of small cell lung cancer (SCLC) pts)
  • Strong female predominance
  • Most common neurologic syndromes
  • Subacute cerebellar ataxia • Limbic encephalitis
  • Encephalomyelitis
  • Opsoclonus myoclonus
  • Subacute sensory neuronopathy
  • Lambert-Eaton myasthenic syndrome
90
Q

How do paraneopl. syndromes present

A

Subacute worsening over weeks to months

• May be presenting symptom of underlying malignancy

Initial cancer screening may be negative

Neuro symptoms occur when malignancy is at a limited stage due to effective anti-tumor immune response

Patientshavemorefavorableoncologicaloutcome

91
Q

Pathogeneis for paraneoplastic syndromes

A
  • Presumably induced against tumor cell antigens • Cross-reac twith neuronal cell antigens
  • Pathogenesis

Hypothesized that some visceral cancers express certain neural antigens; immune system recognizes these antigens as foreign and mounts an immune response against them

Antibodies identified in some paraneoplastic syndromes

92
Q
  • Progressive ataxia, dysarthria, nystagmus, vertigo, diplopia, titubation
  • Associated with ovarian cancer (80%) or breast cancer (10%)
  • Antibody to Purkinje cells
  • Purkinje cell antibody type-1 (PCA-1 or anti-Yo)
A

Subacute cerebellar ataxia: symptom of paraneoplastic syndrome

93
Q

• Clinical: muscle weakness, especially in the legs, that improves with testing on exam

  • Extraocular muscles are spared
  • Antibodies to P/Q-type voltage-gated calcium channels

• Leads to decreased acetylcholine release • Most commonly associated with SCLC

A

Lambert Eaton Myasthenic Syndrome

94
Q

Embryonal epitheilium, in posterior fossa with primitive neuroectoderm neoplasm

S/S ataxia and increased intracranial pressure. Drop mets

A

meduloblastoma

Neuropysch (18 decks)

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