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NMSP Exam I > Tumors (Martin) > Flashcards

Tumors (Martin) Flashcards

1
Q

Intracranial tumors typically arise where in the kids?

A

posterior fossa; infratentorial

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2
Q

Intracranial tumors typically arise where in adults?

A

cerebral hemispheres; supratentorial

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3
Q

If an adult presents with new onset seizures what should you suspect?

A

a brain tumor

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4
Q

What are the 4 histological parameters of gliomas?

A

Nuclear atypia
Mitosis
Microvascular proliferation
Necrosis

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5
Q

Astrocytomas are usually what type of lesions?

A

White matter lesions

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6
Q

The two (MAIN) type of astrocyte tumors?

A

Astrocytomas

in adults - infiltrating astrocytoma
in kids - pilocytic astrocytoma

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7
Q

Infiltrating astrocytoma

A

grade II to IV; mostly adults (80%); presents with seizures, headaches and focal neurological deficits;
IDH-mutant is good prognosis
IDH-wild type is a bad prognosis
confirmatory test with IDH1 R132H mutant protein (90%)

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8
Q

Which marker in infiltrating astrocytoma indicative of a bad prognosis?

A

IDH-wild type

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9
Q

What is the confirmatory test for infiltrating astrocytoma?

A

IDH1 R132H mutant protein (90%)

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10
Q

Pilocytic astrocytoma

A

well circumscribed cystic with a mural nodule tumor of astrocytes in kids located in the cerebellum; IAA 1549 BRAF gene; resection is the only treatment

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11
Q

Characteristics of a pilocytic astrocytoma?

A

has a biphasic pattern; hair-like cells with bipolar processes; Rosenthal fibers and will be GFAP+

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12
Q

Name of tumor that has a biphasic pattern; hair-like cells with bipolar processes; Rosenthal fibers and will be GFAP+?

A

pilocytic astrocytoma

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13
Q

What is the treatment for pilocytic astrocytoma?

A

resection only

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14
Q

What is the most common primary brain neoplasm?

A

Glioblastoma (GBM)

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15
Q

What are the two types of glioblastomas (GBM)?

A

Primary - older patients; IDH - wild type and TERT & EGFR mutations
Secondary - younger patients; IDH1 and TP53 mutation (R132H had better prognosis than IDH - wild type)

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16
Q

Tumor that appears to have hypodense central necrosis on imaging?

A

Glioblastomas (GBM)

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17
Q

What are the 3 histological hallmarks of glioblastomas?

A
  1. Necrosis - serpentine pattern of necrosis
  2. Pseudo-palisading
  3. Vascular/endothelial proliferation
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18
Q

A tumor with these 3 histological hallmarks:
1. Necrosis - serpentine pattern of necrosis
2. Pseudo-palisading
3. Vascular/endothelial proliferation

A

Glioblastomas (GBM)

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19
Q

Tumor of oligodendrocytes?

A

Oligodendroglioma

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20
Q

Oligodendroglioma

A

white matter tumor in older patients; neurological complaints for several years; will see calcifications, “fried eggs” and “chicken wire”
IDH1 & IDH2 - favorable prognosis (90%)
1P19Q loss - favorable prognosis
anaplastic - poor prognosis

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21
Q

What are the genetics associated with favorable prognosis in Oligodendrogliomas?

A

IDH1 & IDH2 - favorable prognosis (90%)
1P19Q loss - favorable prognosis

22
Q

What is indicative of a poor prognosis in Oligodendrogliomas?

A

anaplastic - poor prognosis

23
Q

Tumor that has shows calcifications, “fried eggs” and “chicken wire”?

A

Oligodendroglioma

24
Q

Tumor of ependymal cells?

A

Ependymoma; seen in kids and discretely around the 4th ventricle

25
Q

Ependymoma

A

Tumor of ependymal cells seen in kids and discretely around the 4th ventricle; will see ependymal rosettes (true rosettes - more diagnostic); there are 3 types:
1. Anaplastic
2. Mxyopapillary
3. Subependymoma

26
Q

What is very diagnostic of an ependymoma?

A

true rosettes - tubular structure with central canal; more diagnostic than perivascular rosettes

27
Q

What are the 3 types of ependymomas?

A
  1. Anaplastic (less common)
  2. Mxyopapillary - film terminale
  3. Subependymoma - lateral or 4th ventricle (causes obstructive hydrocephalus)
28
Q

Which type of ependymoma is typically known to cause obstructing hydrocephalus?

A

Subependymoma - because it is located around the lateral or 4th ventricle - can cause obstructive hydrocephalus

29
Q

Which type of ependhymoma is typically known to case lower back pain?

A

Mxyopapillary - because it is located near the film terminal of the spinal cord

30
Q

What are the two types of choroid plexus tumors?

A
  1. Choroid plexus papilloma - children in lateral ventricles
  2. Colloid cyst - young adults attached to roof of 3rd ventricle (obstruct foramen of mono)
31
Q

Gangliogliomas typically affects which part of the cerebral?

A

temporal lobes; mixed with mature neuronal and glial cells; present as seizures and medical refractory epilepsy

32
Q

What is the most common pediatric brain tumor?

A

Medulloblastoma

33
Q

What are the 4 types of medulloblastoma?

A
  1. WNT (BEST prognosis) - older kids with chromosome 6 B-catenin mutation (100% 5 yr survival)
  2. SHH - infants and young adults with MYCN mutation
  3. Group 3 (WORST prognosis) - MYC and 17(I17q) mutation
  4. Group 4 I17q with NO MYC mutation - poor prognosis but better than group 3
34
Q

Which type of medulloblastoma has the worst prognosis and what’s its genetics?

A

Group 3 (WORST prognosis) - MYC and 17(I17q) mutation

35
Q

Which type of medulloblastoma has the best prognosis and what’s its genetics?

A

WNT (BEST prognosis) - older kids with chromosome 6 B-catenin mutation; 100% 5 yr survival

36
Q

Hallmarks of medulloblastomas

A

located at the midline (cerebellum) and will be “drop Metz” throughout the CSF because it is gray friable (tends to fall apart); will also see “cell wrapping”

37
Q

Which intracranial tumors are located at the midline?

A

medulloblastoma (cerebellum) and germ cell tumors (pineal)

38
Q

Atypical teratoid/Rhabdoid tumors

A

located in the posterior fossa; kids under 3 yrs (HIGHLY aggressive - survival <1 yr); will have +EMA and Vimentin and “rhabdoid cells

39
Q

Primary CNS lymphoma

A

seen in immunosuppressed (AIDs) individuals or as a latent infection with EBV; all in B-cell origin so CD20+; will see “hooping”

40
Q

What is the most common type of pineal tumor?

A

Germinoma; male predominance; seen in the first two decades of life and seen in Japanese patients (10%)

41
Q

Tumor of the meninges?

A

Meningioma

42
Q

Meningioma

A

benign tumor of the meninges that compresses the brain (does NOT invade brain parenchyma) and has the potential to penetrate the bone; NF2 mutation on chromosome 22q22; can see calcified psammoma bodies; can be seen in pregnancy and regress after delivery

43
Q

Which tumor has been seen to produce symptoms during pregnancy and then have a relief of symptoms after delivery?

A

Meningioma

44
Q

Mutation of Meningiomas?

A

NF2 mutation on chromosome 22q22

45
Q

Neuroblastoma

A

common extracranial solid tumor of childhood (18 months of age); arise in the adrenal medulla produce catecholamines - “blueberry muffin baby”; presents with large abdominal masses, fever, and possible weight loss; spreads via blood to liver, lungs, bone marrow and bones (also brain)

46
Q

Which childhood tumor is associated with “blueberry muffin baby”?

A

Neuroblastoma; arise in the adrenal medulla produce catecholamines

47
Q

Which two paraneoplastic syndromes can contribute to limbic encephalitis?

A
  1. small cell carcinoma of the lung - ANNA-1 antibody
  2. Ovarian teratoma - NMDA receptor
48
Q

Tuberous Sclerosis Complex

A

one of the familial tumor syndromes; can see “candle gutterings” - wax-like masses building into the ventricles; and cutaneous lesions - angiofibromas and subungal fibromas

49
Q

Which one of the drugs from Segars AED lecture is used for Tuberous Sclerosis Complex?

A

Cannabidiol (Epidiolex)

50
Q

Von Hippel-Lindau Disease

A

a familial tumor syndrome; AD inheritance causes POLYCYTHEMIA (down regulation of HIF1 which regulates VEGF); hemangioblastomas of the CNS; cysts in the pancreas,liver and kidneys, renal cell carcinoma and pheochromocytoma

51
Q

Main symptom of Von Hippel-Lindau Disease?

A

POLYCYTHEMIA (down regulation of HIF1 which regulates VEGF)

52
Q

What are the 3 peripheral nerve sheath tumors?

A
  1. Schwannoma - NF2 mut; causes acoustic neuroma CN VIII; does NOT invade nerve
  2. Neurofibroma - NF-1 mut; does invade nerve
  3. MPNST - malignant - NF-1 mut

NMSP Exam I (18 decks)

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