Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

NMSP Exam 1 > CNS Tumors > Flashcards

CNS Tumors Flashcards

1
Q

Which tumors are common in children?

A

Pilocytic astrocytoma, glioblastoma, all other astrocytomas, ependymoma, oligodendrogliomas, embryonal including medulloblastomas, craniopharyngioma, germ cell tumors

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

What are the most common pediatric tumors?

A

Embryonal/medulloblastomas and pilocytic

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

Where are tumors usually located in adults?

A

Supratentorial

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

Where are tumors usually located in children?

A

Infratentorial, cerebellum, and brainstem

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

Which tumor is more common in adults?

A

Glioblastoma (new onset of seizure in adult think tumor)

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

What are common pediatric brain tumors?

A

Astrocytomas, ependymoma, choroid plexus (papilloma, carcinoma), embryonal (medulloblastoma, PNET, AT/RT), neuronal and glial (ganglioglioma, DNET), non-neuroectodermal (craniopharyngioma, germ cell tumor)

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

What is a grade I tumor?

A

Low proliferative potential, possibility of cure by resection

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

What is a grade II tumor?

A

Infiltration, despite low proliferative potential likely to recur; some grade II progresses to higher grade; cytological atypia (ex. Diffuse astrocytoma); 1 parameter

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

What is a grade III tumor?

A

Need to be treated with radiation and chemo; anaplasia and mitoses (ex. Anaplastic astrocytoma); 2 parameters

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

What is a grade IV tumor?

A

Rapid pre and post-op evaluation, fatal outcome, microvascular proliferation and/or necrosis (contain 3-4 parameters)

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

What are the 4 histological parameters for gliomas?

A

Nuclear atypia, mitoses, microvascular proliferation, necrosis

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

What are examples of grade I astrocytomas?

A

SEGA and pilocytic astrocytoma

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

What are examples of grade II astrocytomas?

A

Diffuse astrocytoma, PXA, pilomyxoid astrocytoma

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

What are examples of grade III astrocytomas?

A

Anaplastic astrocytoma

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

What are examples of grade IV astrocytomas?

A

Glioblastoma (GBM), giant cell GBM, gliosarcoma

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

Grade I astrocytomas usually occur at what age?

A

Usually first decade

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

Grade II astrocytomas usually occur at what age?

A

Usually 3rd-4th decade

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

Grade III astrocytomas usually occur at what age?

A

Usually 5th decade

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

Grade IV astrocytomas usually occur at what age?

A

Usually 6th decade and beyond

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

Elderly individual with well differentiated lesion should cause what?

A

Should raise concern of sampling

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

What are the histological subtypes of medulloblastoma?

A

Classic (70% of MBs), desmoplastic (PTCH1 gene on Chr 9 which has a better prognosis), Anaplastic, large cell, MB with extensive nodularity (MBEN)

22
Q

What are the molecular subtypes of medulloblastoma?

A

WNT, SHH, Grp 3 (non-Wnt/non-Shh)-MYC, group 4 (non-wnt/non-shh)-MYCN,CDK6

23
Q

PTCH1 gene is associated with what?

A

Nevoid BCC syndrome (NBCCS aka Gorlin syndrome)

24
Q

APC gene is associated with what?

A

Familial adenomatous polyposis; part of WNT

25
Q

What is TP53 associated with?

A

Li-Fraumeni syndrome; TP53 part of SHH

26
Q

What are some differential diagnoses for medulloblastoma?

A

Ependymoma, pilocytic astrocytoma, high grade infiltrating astrocytoma, atypical teratoid/rhabdoid tumor, metastatic carcinoma in adult pts

27
Q

What are some characteristics of ependymoma?

A

Perivascular pseudorosettes, but less cellular and more fibrillar than medulloblastoma

28
Q

What are some characteristics of pilocytic astrocytoma?

A

Cystic (medulloblastoma solid), biphasic architecture, lower cellularity

29
Q

What are some characteristics of high grade infiltrating astrocytoma?

A

Usually more pleomorphic than medulloblastoma

30
Q

What are some characteristics of AT/RT?

A

<2yo, large cells with paranuclear filamentous inclusions; problem with large cell medulloblastoma

31
Q

What are some familial syndromes associated with pheochromocytoma and extra adrenal paragangliomas?

A

Neurofibromatosis type 1 and Von hippel lindau

32
Q

What gene is associated with neurofibromatosis type 1?

A

NF1

33
Q

What is the associated lesion for neurofibromatosis type 1?

A

Pheochromocytoma

34
Q

What are some other features related to neurofibromatosis type 1?

A

Neurofibromatosis, cafe au lait spots, optic nerve glioma

35
Q

What gene is associated with Von Hippel Lindau syndrome?

A

VHL

36
Q

What is the associated lesion for Von Hippel Lindau syndrome?

A

Pheochromocytoma, paraganglioma (uncommon)

37
Q

What are some other features related to Von Hippel Lindau syndrome?

A

RCC, hemangioblastoma, pancreatic endocrine neoplasm

38
Q

What are some familial cancer syndrome that have cutaneous manifestations?

A

Nevoid basal cell carcinoma syndrome, Cowden syndrome, neurofibromatosis I and II, tuberous sclerosis

39
Q

What is the genetic profile of Nevoid BCC syndrome?

A

AD inheritance, located on Chr 9q22 and associated gene is PTCH

40
Q

What is the normal function and manifestation loss in Nevoid BCC syndrome?

A

Developmental patterning gene; multiple BCC, medulloblastoma, jaw cysts

41
Q

What is the genetic profile for Cowden syndrome?

A

AD inheritance, Chr 10q23, associated gene is PTEN

42
Q

What is the normal function and manifestation of loss for Cowden syndrome?

A

Lipid phosphate; benign follicular appendage tumors (trichilemmomas), internal adenocarcinoma (often breast or endometrial)

43
Q

What is the normal function and manifestation of loss for neurofibromatosis I?

A

Negatively regulates RAS signaling; neurofibromas

44
Q

What is the normal function and manifestation of loss for neurofibromatosis II?

A

Integrates cytoskeletal signaling; neurofibromas and acoustic neuromas

45
Q

What is the genetic profile for tuberous sclerosis?

A

AD inheritance, Chr 9q34 and 16p13, genes include TSC1 (hamartin) and TSC2 (tuberin)

46
Q

What is the normal function and manifestation of loss for tuberous sclerosis?

A

Work together in a complex that negatively regulates mTOR; angiofibromas, intellectual disability

47
Q

What CNS tumors are associated with Cowden syndrome?

A

Dysplastic gangliocytoma

48
Q

What CNS tumors are associated with Li Fraumeni syndrome?

A

Medulloblastomas

49
Q

What CNS tumors are associated with Turcot syndrome?

A

Medulloblastoma or glioblastoma (mutations in APC or mismatch repair genes)

50
Q

What CNS tumors are associated with Gorlin syndrome?

A

Medulloblastoma (mutations in PTCH gene resulting in upregulation of shh pathway)

NMSP Exam 1 (8 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions