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Brain tumors of childhood Flashcards

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1
Q

What are the brain tumors of childhood (5)

A

1) Pilocytic Astrocytoma
2) Craniopharyngioma
3) Choroid Plexus tumors
4) Medulloblastoma
5) Ependymoma

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2
Q

What are the Grade 1 tumors of childhood (3)

A

1) Pilocytic Astrocytoma
2) Craniopharyngioma
3) Choroid plexus papilloma

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3
Q

What is the Grade 2 tumor of childhood?

A

Ependymoma

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4
Q

What is the Grade 3 tumor of childhood?

A

Choroid plexus carcinoma

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5
Q

What is the Grade 4 tumor of childhood?

A

Medulloblastoma

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6
Q

What is the definition of Grade 1?

A

Benign, slow growing, normal-looking histology. Long term survival expected. Rare in adults

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7
Q

What is the definition of Grade 2?

A

Relatively slow growing, some tumor spread, recurs after removal often higher grade. Slightly abnormal histology

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8
Q

What is the definition of Grade 3?

A

Malignant, tumor spreads, abnormal histology, recurs after removal often higher grade

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9
Q

What is the definition of Grade 4?

A

Malignant, grows quickly, spreads widely, very abnormal histology, angiogenesis and necrosis present

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10
Q

What is the #1 brain tumor that’s not of neuroepithelial origin? What’s the embryological structure that forms it?

A

Craniopharyngiomas are formed from Rathke’s pouch (epithelial origin). Most are suprasellar (above the sella turcica) but can have an intrasellar component

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11
Q

Where do pilocytic astrocytomas form?

A

Centralized location and Periventricular (mostly 3rd ventricle). Also in cerebellum

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12
Q

Pilocytic astrocytomas form in which trisomies?

A

Trisomy 7 and 8

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13
Q

What is the gross appearance of pilocytic astrocytomas?

A

Well circumscribed cystic lesion with a mural nodule

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14
Q

What is the histology of pilocytic astrocytoma?

A

Spindly and elongated astrocytes with Rosenthal fibers

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15
Q

What are Rosenthal fibers?

A

Thick eosinophilic corkscrew bundles on H&E

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16
Q

What is the marker for pilocytic astrocytoma

A

GFAP

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17
Q

What is the gross appearance of craniopharyngioma?

A

Well defined lobulated mass with cyst formation. “Car oil” is present in the cyst

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18
Q

What is the histology of craniopharyngioma?

A

Stratified squamous epithelium with keratin and CALCIFICATIONS

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19
Q

In which ventricles do choroid plexues tumors grow?

A

Lateral and fourth ventricles

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20
Q

What genetic syndrome has a propensity to develop choroid plexus tumors?

A

Li-Fraumeni syndrome (p53 mutation)

21
Q

What is the histology of choroid plexus adenomas?

A

Fibrovascular connective tissue fronds (blood vessel within connective tissue) and a single layer of columnar epithelium

22
Q

What % of choroid plexus tumors are carcinomas instead of papillomas?

A

1% are carcinomas

23
Q

What are the markers for choroid plexus tumors?

A

Epithelial membrane antigen and carotenes

24
Q

What is a common presenting sign for pts with choroid plexus tumors?

A

hydrocephalus

25
Q

What is the #1 solid brain tumor of kids?

A

Medulloblastoma

26
Q

When do classic and neuroblastic medulloblastomas occur?

A

in kids under 10 years old (classic or neuroblastic)

27
Q

What is the other kind of medulloblastoma? When does it occur?

A

Desmoplastic. 12-20 years old

28
Q

Where do classic medulloblastomas grow?

A

Mid cerebellum (vermis)

29
Q

Where do desmoplastic medulloblastomas grow?

A

Lateral cerebellar hemispheres

30
Q

What is the chromosomal abnormality associated with medulloblastoma?

A

Isochromosome 17q (both 17q’s from either mom or dad but not both); also can have 1q mutations

31
Q

What is the gross appearance of medulloblastoma?

A

Gray nodular mass that is WELL defined

32
Q

What is the histology of classic medulloblastoma?

A

Dense undifferentiated cells with little to no cytoplasm (small round blue cells)

33
Q

What is the histology of neuroblastic medulloblastoma?

A

More differentiated cells with elongated nuclei and rosettes

34
Q

What is the histology of desmoplastic medulloblastoma?

A

Most differentiated cells with germinal centers

35
Q

What are the markers from medulloblastoma?

A

Chromogranin, synaptophysin, neurofilaments (remember it’s neural not glial so no GFAP)

36
Q

What is the prognosis for medulloblastoma?

A

Good prognosis even though it is Grade 4

37
Q

What are the two types of ependymomas?

A

Intratentorial and Spinal

38
Q

When do infratentorial ependymomas occur?

A

under 10 years old

39
Q

When do spinal ependymomas occur?

A

pt in their 30s

40
Q

Where do infratentorial ependymomas occur?

A

4th ventricle near pons

41
Q

Where do spinal ependymomas occur?

A

Cauda equina

42
Q

What is the mutation associated with ependymomas?

A

22q mutation

43
Q

What is the gross appearance of ependymomas?

A

Well demarcated mass with cysts and possible calcifications

44
Q

What is the histology of ependymomas?

A

Homogeneous cells with cilia in Pseudorosettes and true rosettes

45
Q

What is the pseudorosette in ependymoma called?

A

Perivascular pseudorosette (tumor cells around a central blood vessel)

46
Q

What are the rosettes called in ependymoma?

A

True ependymal rosettes - tumor cells around an empty space (little ventricle)

47
Q

What is a common presenting sign with ependymoma?

A

Hydrocephalus due to obstruction

48
Q

What % of ependymomas are supratentorial?

A

40%

Brain Tumors (2 decks)

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