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Neuromusculoskeletal & Psychiatry II Exam #2 > CNS Tumors > Flashcards

CNS Tumors Flashcards

1
Q

Which patients are more likely to have supratentorial tumors?

Which patients are more likely to have infratentorial tumors?

A

Supratentorial - adults

Infratentorial - kids

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2
Q

What is meant by the following tumor grades:

Grade I

Grade II

Grade III

Grade IV

A

Grade I: low proliferative potential, possibly of cure by resection.

Grade II: infiltrative, despite low potential, are likely to recur. Possess cytological atypia.

Grade III: require radiation and chemo. Include anaplasia and mitoses (anaplastic astrocytoma).

Grade IV: rapid pre- and post-op evolution with fatal outcome. Microvascular proliferation and/or necrosis.

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3
Q

What are the 4 histological parameters of Gliomas?

How many parameters exist in the following:
Grade II
Grade III
Grade IV

A

Nuclear atypia
Mitoses
Microvascular proliferation
Necrosis

Grade II - 1 parameter
Grade III - 2 parameters
Grade IV - 3 or 4 parameters

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4
Q

What ages correlate to the following Astrocytomas?

Grade II
Grade III
Grade IV

A

Grade II - 3rd-4th decade
Grade III - 5th decade
Grade IV - 6th decade or more

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5
Q

Astrocytomas are usually made of what?

What does signs does a brainstem astrocytoma have?

A

White matter

Long tract and brainstem signs (CN VI dysfunction)

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6
Q

What grades does a Pilocytic Astrocytoma have?

Which ages are most common?

Where is the tumor found?

What is the genetic predisposition?

What is the gross appearance of the tumor?

A

Grade I and IV

First 2 decades of life

Cerebellum or cerebral hemispheres

NF1, especially optic n. tumors (functional loss of neurofibromin in tumor)

Well-circumscribed, often cystic with a mural nodule

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7
Q

What can be found within a Pilocytic Astrocytoma? (5)

What stain is positive?

A

Biphasic pattern: loos glial cells with cystic changes and dense piloid tissue.

Hair-like cells with bipolar processes.

Rosenthal fibers

Eosinophilic granular bodies (EGBs)

Optic n. lesions

GFAP+

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8
Q

What is the most common primary brain neoplasm?
What grades can it have?

Where in the brain are they most commonly found?

A

Glioblastoma
Grade IV/IV

Throughout the brain

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9
Q

What is the difference between primary and secondary glioblastomas? What molecular changes are seen in each?

A

Primary: older patients and no precursor lesion.
-EGFR and PTEN

Secondary: preceded by lower grade lesion (TP53).

  • IDH1
  • R132H mutation has a better prognosis!
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10
Q

What is the appearance of Glioblastoma?

A

Contrast ring enhancing with hypodense central necrosis.

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11
Q

What are the 3 histological hallmarks of Glioblastoma?

A
  1. Necrosis: “Serpentine” pattern of necrosis in hypercellular areas.
  2. Pseudo-palisading of cells around necrosis.
  3. Vascular/endothelial proliferation.
    - may produce a “glomeruloid body”
    - VEGF is produced by alignant astrocytes in response to hypoxia.
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12
Q

Who is affected most by Oligodendrogliomas?

Where are these tumors located usually?

What features are seen on histology?

What grades are they?

Which genetic changes might be seen? What is the prognosis?

A

Adults

Cerebral hemispheres

Calcification
Perinuclear halos - “fried eggs” (artifact)
Delicate anastomosing capillaries - “chicken wire”

Grade II/IV

IDH1 and IDH2 (90%) - favorable
1P19Q loss - favorable

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13
Q

What grade is Anaplastic Oligo?

What is the prognosis?

A

Grade III/IV

Poor prognosis, similar to glioblastoma

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14
Q

Ependyloma is found in which patients?

What sites does it affect in the brain most?

What grade is it?

What is diagnostic?

A

First 2 decades of life

4th ventricle: discrete, exophytic, enhancing
Supratentorial: cystic and paraventricular

Grade III - usually supratentorial, mitoses, microvascular proliferation, necrosis; clear cell variant

Ependymal rosettes (true rosettes) are more diagnostic than perivascular rosettes

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15
Q

Which patients are more likely to develop a Choroid Plexus Papilloma?

Where does it occur?

What does it cause?

What would be on the DDx?

A

Children

Lateral ventricles

Hydrocephalus: obstruction
-increased CSF production

DDx: choroid plexus papilloma in adult vs. metastatic carcinoma

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16
Q

Who is most likely to develop a Colloid Cyst of 3rd Ventricle?

Where does it occur? What does it cause?

What is the outcome?

A

Young adults

Attached to roof of 3rd ventricle -> obstruct foramen of Monro = non-communicating hydrocephalus

Can be rapidly fatal! Can also be positional.

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17
Q

Who is most likely to develop Medulloblastoma?

What grade is it?

Where do they occur in the brain?

What is seen on histology?

What is the treatment?

A

Children (20% of tumors in kids)

Grade IV/IV

Cerebellum, midline

Sheets of anaplastic cells, abundant mitoses, Homer-Wright rosettes

Total excision and XRT = 75% survival at 5 years; very radiosensitive sensitive

18
Q

What are the 4 molecular groups and prognoses of Medulloblastoma?

A

WNT: older kids, monosomy chr. 6, B-catenin = 5-year survival

SHH: infants and young adults, MYCN = prognosis is intermediate between WNT and groups 3 and 4

Group 3: MYC and 17(I17Q) = worst prognosis

Group 4: I17Q, no MYC, +/-MYCN = poor prognosis

19
Q

What grade is Atypical Teratoid/Rhabdoid tumor?

Where does it occur?

What molecular stains are used?

What cells are seen on histology?

What molecular changes are seen?

What is the outcome?

A

Grade IV/IV

Posterior fossa or supratentorial

+ EMA, Vimentin

Rhabdoid cells: eosinophilic cytoplasm with sharp borders and eccentric nucleus; resembles Rhabdomyosarcoma

> 90% have chr. 22 HSNF5/INI1

If < 5 y/o - highly aggressive; usually < 1 year survival

20
Q

Who is most likely to develop Primary CNS Lymphoma?

Where do they occur most?

What is the cell origin? What marker is positive?

It is often latently infected with what virus?

What is the morphology?

What suggests a CNS primary lesion?

A

Immunosuppressive individuals - AIDS

Usually multifocal and periventricular

B-cells and CD20+

EBV

Accumulate around vessels

“Hooping” = cells separated by reticulin and silver stain is chaacteristic of CNS primary

21
Q

Where do Germ Cell tumors occur?

What ethnicity is most common?

What should be ruled-out?

What is the prognosis?

What markers are used to track response to treatment?

A

Along the midline
-pineal gland (males mostly) and suprasellar

Japanese

Mets of gonadal germ cell to CNS is common, so must be R/O

Good - excellent response to XRT and chemo

AFP and bHCG

22
Q

What is a Pinecytoma?
Who is at a greater risk?
What is the differentiation?

What is a Pineblastoma?
Who is at a greater risk?
What is the differentiation?
What gene is implicated often?

A

Pinecytoma: well-differentiated; low-grade usually adults

Pineblastoma: poorly-differentiated; high-grade with mitoses and necrosis; usually kids
-RB gene mutation

23
Q

What age is most common to develop a Meningioma?

Where does it spread?

What induces the tumor?

What is the gene association?
Which one is associated with a lower grade histology?

What is it composed of?
What stain is positive?

A

> 3rd decade

Penetrates bone - hesitant to invade brain, so will compress it instead

Radiation

NF2
TRAF7 - lower grade and more stable

Dura based - meningothelial cell origin of arachnoid
EMA+

24
Q

What are histological findings of a Meningioma?

What is meant by En Plaque?

The “secretory subtype” is determined by what positive stain? What mutations does it have?

What are the differences between Atypical II/IV and Anaplastic II/IV?

A

Whorled clusters of monotonous cells and Psammoma bodies

Sheet-like spread of tumor along surface of dura

PAS+; TRAF7 and KLF4 mutations

Atypical II/IV: > 4 mitoses /10hpf
-Clear and Choroid II/IV more aggressive

Anaplastic II/IV: > 20 mitoses/10hpf; resembles sarcoma
-Papillary and Rhabdoid subtypes

25
Q

What is the association with Meningioma and pregnancy?

A

They are slightly more common in women (3:2)

Approx. 70% express PR (some express ER)

About half of women found relief of symptoms within 1 week post-partum

26
Q

What are 3 major sites that metastasize to the brain?

What are 2 more uncommon tumors, but often go to brain?

Hemorrhage is associated with which primary tumors? (4)

Meningeal metastasis are common. What is the site of intraparenchymal metastases?

What is carcinomatosis? Which 2 tumors are commonly implicated?

A

Lung (35%), breast (20%), melanoma (10%)

Choriocarcinoma (rare) but mets to brain
Renal cell carcinoma lead to late mets

Melanoma, Choriocarcinoma, RCC and lung

Gray/white matter junction

Tumor nodules spread along the surface or brain and cord; lung and breast most commonly

27
Q

What antibody is positive in Subacute Cerebellar Degenration paraneoplastic syndrome?

What is the process of the syndrome?

Which cancers is it associated with?

A

PCA-1

Destruction of Purkinjes, gliosis and mild chronic inflammatory infiltrate

Women with ovarian, uterine or breast cancer

28
Q

Which of the following antibodies/receptors are associated with which primary process?

ANNA-1
NMDA-receptor
VGKC-somplex antibody

What do they cause generally?

A

ANNA-1: small cell lung cancer (CNS and PNS)
NMDA-receptor: ovarian teratomas (hippocampus)
VGKC-complex antibody: peripheral neuropathy (VG K+ channels)

Limbic encephalitis - these symptoms often appear before malignancy is suspected

29
Q

Eye movement disorders, like opsoclonus, is a paraneoplastic syndrome associated with which malignancy?

A

Neuroblastoma in kids

30
Q

What paraneoplastic syndrome affects voltage-gated Ca++ channels?

A

Lambert-Eaon myasthenic syndrome

31
Q

NF1 is associated with which lesion?

Inheritance and prevalence:

What are 4 other associations?

A

Pheochromocytoma

AD, 1/3000

Neurofibromas of peripheral nerves
Cafe-au-lait spots
Optic n. glioma
Lisch nodules - pigmented nodules of iris

32
Q

VHL is associated with which 2 lesions?

What are 3 other associations?

A

Pheochromocytoma, Paraganglioma (rare)

Renal cell carcinoma
Hemangioblastoma
Pancreatic endocrine neoplasm

33
Q

What is the normal function of NF1 gene?

What is the normal function of NF2 gene?

A

NF1: negatively regulates RAS signaling (neurofibromin)

NF2: integrates cytoskeletal signaling (merlin)

34
Q

What is the mutation association with the following:

Cowden syndrome
Li-Fraumeni syndrome
Turcot syndrome
Gorlin syndrome

A

Cowden syndrome: PTEN -> IK3/AKT
Li-Fraumeni syndrome: TP53
Turcot syndrome: APC
Gorlin syndrome: PTCH -> upregulation of SHH

35
Q

What is the inheritance of TSC? What are the symptoms?

What is the protein and location of TSC1 and TSC2?

What are the major associated tumors? (4)

A

AD (1/6000) - seizures, autism, MR

TSC1: chr. 9Q34 - Hamartin
TSC2: chr. 16P13.3 - Tuberin

Hamartomas
Retinal angiomyolipomas
Cardiac rhabdomyomas
Cutaneous lesions: Shagreen patches (localized cutaneous thickenings) and Ash-leaf patches (hypopigmented areas)

36
Q

What are the molecular consequences of VHL disease?

What is the inheritance?

What are classic conditions/tumors seen? (5)

A

VHL mutation downregulates HIF-1, which regulates expression of VEGF

AD

Polycythemia
Hemangiomas of CNS (cerebellum and retina)
Cysts in pancreas, liver and kidneys
Renal cell carcinoma
Pheochromocytoma
37
Q

NF2 is associated with which lesion?
Which 2 other lesions are of increased prevalence?

Inheritance and prevalence:

A 
Bilateral Schwannomas (CN VIII)
Meningiomas and Ependymomas

AD, 1/40-50K

38
Q

Where do Schwannomas occur? What marker is positive?

What is an associated tumor?

What gene is defective?

What is seen in the Antoni A variant?

A

Cerebellar pontine angle - S100+

Acoustic neuroma = CN VIII dysfunction

NF2 - loss of merlin

Verocay bodies = palisading nuclei around “nuclear free zones”

39
Q

What appearance is associated with Plexiform Neurofibroma? What is a possible complication?

What is the gene association?

A

“Bag of worms” - may cause malignant transformation to MPNST

NF1

40
Q

What is a malignant peripheral nerve sheath tumor (MPNST)?

What is a subtype?

A

A tumor that arises in 85% ofhigh-grade tmors and about 1/2 of NF1 patients

Triton tumor - Rhabdomyoblastic differentiation

Neuromusculoskeletal & Psychiatry II Exam #2 (15 decks)

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