Brainscape's Knowledge GenomeTM


Top Flashcards (Certified)
All Flashcards

Boards > BRAIN TUMORS > Flashcards

BRAIN TUMORS Flashcards

(95 cards)

Start Studying
1
Q

most common solid malignancy in children and most common malignancy overall in kids

A

solid: CNS, overall: leukemia with CNS next

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

gender distribution in CNS tumors

A

M >F

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

name the gene abnormality and CNS leison: neurofibromatosis type 1

A

NF1, LGG (optic glioma, brainstem)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

name the gene abnormality and CNS leison: Neurofibromatosis type 2

A

NF2, bilateral acoustic schwannomas, meningiomas, and ependymomas

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

name the gene abnormality and CNS leison Tuberous Sclerosis

A

TSC1 and TSC2, subependymal giant cell astrocytoma (SEGA)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

name the gene abnormality and CNS leison: Li Fraumeni

A

TP53, malignant glioma, choroid plexus carcinoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

name the gene abnormality and CNS leison Gorlins (nevoid basal cell carcinoma syndrome)

A

PTCH, medulloblastoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

name the gene abnormality and CNS leison (familial adenomatous polyposis aka Gardners or Turcots)

A

APC, medulloblastoma, malignant glioma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

name the gene abnormality and CNS leison rhabdoid tumor predisposition syndrome

A

SMARCB1 and SMARCB4 (aka INI-1), AT/RT

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

name the gene abnormality and CNS leison retinoblastoma (gremlin)

A

RB1, trilateral retinoblastoma aka unilateral or bilateral + pineoblastoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

name 3 key other findings in NF1

A

cafe au last spots, lisch nodules, axillary freckling

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

increased risk of what besides CNS tumors in NF2

A

cataracts, seizures

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

increased risk of what in tuberous sclerosis (Besides CNS lesions)

A

skin and renal growths

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

key risks in Li Fraumeni

A

numerous cancers at younger ages- breast, sarcoma, adrenal cortical carcinoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

key other tumor in gorlins

A

basal cell carcinoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

other key things to know about rhabdoid tumor predisposition syndrome

A

rhqbdoid renal tumors, schwannomatosis, usually less than 1 year at diagnosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

when is pineoblastoma often dx in trilateral rB

A

after RB but before age 5

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

what fraction of peds brain tumors are infratentorial

A

2/3

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

describe symptoms of a tumor int his location: frontal lobe

A

personal8ty, brocas (decreased motor speech), seizures

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

what are symptoms in temporal leison

A

seizures, poor memory, language comprehension (wernickes)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

symptoms if tumor in parietal lobe

A

decreased sense of touch/pain, poor spatial and visual perception, poor interpretation of language

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

occipital lobe tumor symptoms

A

vision

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

where is the brain tumor if there is autonomic dysfunciton

A

brainstem or hypothalamus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

where is the tumor if consciousness and weakness and motor control with sleep abnormalities

A

thalamus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
what is the most common pediatric CNS tumor and how many of peds brain tumors are this
LGG_ 30%
26
how to make diagnosis of LGG
biopsy or resection with goal of CR. EXCEPTION- NF1 if classic appearance in optic pathway
27
What staging is needed for most LGG and what is 1 exception
generally none. if pilomyxoid astrocytoma, increased risk of mets
28
treatment for SEGA
historically surgery, now Mtor inhibitors like everolimus
29
Diagnostic criteria for NF1 if a kid does not have a parent with NF1 (7)
2 or more of the following; - 6 or more cafe o last, axillary OR inguinal freckles, 2 or more neurofibromas of any time, 1 plexiform neurofibroma, optic pathway glioma, 2 or more iris lunch nodule OR 2 or more choroidal abnormalities, key osseous leison, heterozygous NF1 variant n 50% of apparently normal cells
30
how many criteria are needed t dx NF1 f there's a kid whose parent has the dx
1 or more
31
what % of pts with NF1 will develop LGG
15-20
32
treatment strategy in NF1 LGG
often dont do anything unless in optic pathway with visual acuity decline or other Neuro symptom. then step 1) chemo
33
what treatment is avoided in NF1
radiotherapy and alkylators- high risk of secondary malignancy
34
most common LGG histology
pilocytic astrocytoma
35
describe path of pilocytic astrocytoma
Rosenthal fibers (corkscrew shape, eosinophilic on H and E), low to moderate cellularity with + GFAP
36
most common molecular abnormality in LGG
MAPk abberations
37
non NF1 LGG treatment approach
attempt total resection. then chemo (1 carbo/VCR, 2 Vinblastine, 3 is combo). Radiation works but avoid- developmental
38
recently accepted new standard of care for kids 1 year and older with BRAF V600E mutant LGG
dabrafenib/trametinib
39
define diencephalic syndrome
usually tumor in diencephalon aka hypothalamus an thalamus, usually from LGG. FTT, emaciation, abnormal eye issues, vomiting, hydrocephalus . Would be indication to treat with chemo
40
who is at risk of HG
previous chemo or radiation exposure and patients with the following two syndromes: Li Fraumeni OR FAP
41
diagnosis of HGG and excpetion
biopsy/resection EXCEPT DIPG
42
staging for HGG
spine MRI, do NOT need LP
43
histology of HGGT
HIGHLy cellular, lots of mitosis, vascular proliferation, pseudopalisading necrosis, USUALly gap +
44
genetic finding in most DIPG
histone mutation (H3 K27M)
45
classic triad of DIPG
1. Cranial nerve symptom, 2. long-tract signs, 3. ataxia
46
only known beneficial therapy for DIPG
radiation
47
mean survival of DIPG
10-12 months, all dead by 2 years
48
most common malignant brain tumor of childhood
medulloblastoma
49
where is medulloblastoma located
posterior fossa (cerebellum)
50
M:F ratio in medullo
1.7 to 1
51
increase risk of medulloblastoma in who
Gorlins syndrome (aka nevoid basal carcinoma syndrome) DUE TO PTCH gene mutation in SHH pathway. ALSO in FAP (aka Gardners and turbots due to APC)
52
diagnosis of medulloa
resection (both diagnostic and prognostic), you need LP and spine MRI- usually mets exist
53
when to do staging for medulloa
before surgery or 10-14 days after
54
histology of medulloblastoma
highly cellular, synaptophysin positive
55
4 major molecular subgroups of medullo
1. WNT, 2. SHH, 3. Group 3, 4. group 4
56
which subgroup of medullo has best prognosis
WNT, early 90-100%
57
what subtype of medullo is usually infants
SHH
58
worst prognostic subgroup of medullo
group 3
59
what type of medullo do patients with goblins get
SHH
60
3 key risk factors for medullo that make you High risk
1. Resection-- high risk if >1.5cm cubed of residual tumor (otherwise standard). 2. M Staging- any mets are high risk. 3. histology- bad is anaplastic/large cell
61
standard risk 5 year OS medulloblastoma
75-90%
62
third most common pediatric CNS tumor
ependymoma
63
who has increased risk of ependymoma
NF2
64
staging: ependymoma
must include full spine MRI and LP
65
where is ependymoma and general term youll see on rads report
usually in cerebellum, "patchy heterogenous enhancement"
66
pathology of ependymoma histology
perivascular pseudo rosettes, monomorphic cells with oval nuclei
67
treatment approach ependymoma
maximum resection. if localized, then radiation. chemotherapy has unclear benefit and only used if under 1 year
68
ependymomas outcome
5 year OS is 50-60% but actually keeps declining after that. Long term survival is more like 30%, generally recurs
69
2 circulating tumor markers you can see in CNS germ cell tumors
AFP and beta HCG
70
2 major categories of germ cell CNS tumors
1. Germinoma. Does NOT secrete. better outcome. 2 non-germinomatous germ cell tumor (SECRETES_ worse outcome)
71
where are germ cell tumors more common in the world
Japan and asian countries (up to 15%, more like 2-3% in US)
72
imaging of germ cell tumor
heterogenous and enhancing mass in 1 of the following locations- 1. suprasellar pituitary OR pineal (could be both- doublet or bifocal)w
73
what is parinauds sydnrome
cluster of eye abnormalities in movement and pupil dysfunction, can be from pineal tumor
74
what type of germ cell tumor has elevated beta HCG
choriocarcinoma
75
hat type of germ cell tumor has elevated AFP
yolk sac tumor
76
how to diagnose CNS germ cell tumor
if imaging is in pineal gland and suprasellar region and AFP or bHCG Is very high, that is good enough. otherwise, biopsys
77
staging CNS germ cell tumor
spine MRI and LP
78
three tumors that need full staging
ependymoma, medullo, germ cell tumor
79
treatment approach for germinoma
either neoadjuvant chemo then response based XRT OR XRT alone
80
treatment approach NGGCT
neoadjuvant then XRT
81
Prognosis germinoma
excellent more than 90%
82
associate schwannomatosis with this
ATRT and rhabdoit umor predisposition syndrome
83
where are choroid plexus tumors usually located
lateral ventricles
84
presenting symptoms in craniopharyngioma and location
headache, emesis, visual field cut, endocrinopathies. most common in suprasellar- pituitary
85
long term effects of CNS radiation and timeline
second brain tumor- HGG and meningioma. Usually 7-10 years. risk is less tan 5%
86
long term toxicity of MTX
leukoencephalopathy
87
long term toxicity of cisplat
oto toi, renal
88
long term cyclophosphamide tox
renal, infertility
89
time frame for secondary malignant brain tumor
7-10 yrs
90
2 common types of secondary brain tumors
meningioma and HGG
91
2 syndromes with risks for LGG
NF1 and TS (for SEGA)
92
3 big risks for HGG
Li fraumemi, hx CNS radiation, FAP
93
2 syndromes with risks for medullo
Nevoid basal cell carcinoma, FAP
94
syndrome with risk for ependymoma
NF2
95

Boards (24 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions