Lecture 8 and 12 -- CNS Tumors + Path Flashcards Preview

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Flashcards in Lecture 8 and 12 -- CNS Tumors + Path Deck (32):
1

what is the most common malignancy to the brain?

Metastatic disease -- 50% from the Lung

2

where do most ped tumors occur? where do most adult tumors occur?

Peds -- 75% in Posterior Fossa

Adults -- 75% in cerebral hemispheres

3

WHO grading system (using astrocytoma as an example; describe the progression and associated micropath at each grade)

Grade 1 -- ?

Grade 2 -- Diffuse Astrocytoma -- some atypical cells but no mitotic activity

Grade 3 - Anaplastic Astrocytoma -- Increased cellularity and mitotic activity

Grade 4 - Glioblastoma -- vascular proliferation/endothelial patches, necrosis, pseudopalisading

4

What is meant by the term "diffuse"?

infiltrate the brain as individual cells; wonder out into the brain parenchyma

therefore technically non curable and start as grade 2 tumors

5

Glioblastoma --


pathognomonic micropathology finding?

Psuedopalisading

6

Primary vs secondary glioblastomas?

Primary -- Preseents as glioblastoma, not an evolving precursor lesion.

Secondary -- Evolves from Precursors

7

Glioblastoma --
Median survival and pathogenesis?

Treatment ?

marker to test for?

the most common Primary CNS Malignancy
<12 month median survival. Characteristic rapid progression, significant mass effect, edema

Treatment: Cannot resect
Tx = Radx + Temozolomide


MGMT methylation

8

What is the significance of an MGMT mutation for Glioblastoma treatment?

MGMT negates the effect of Temozolomide
therefore if there is an MGMT methylation, the gene is silenced and patient responds better to Temozolomide therapy

Unmethylated MGMT = negative prognostic factor (irrespective of treatment)

9

Anaplastic oligodendroglioma --

Pathognomonic pathology?

genetic mutation/marker ?

prognostic significance?

Friend Egg with Chicken wire

1p/19q Co Deletion -- positive prognostic factor

10

name 3 low grade gliomas ?

what is the standard therapy?

what is the median survival time?

§ Diffuse astrocytoms -- 5 years
Oligoastrocytoma -- 7.5 years
Oligodendroglioma -- 10 years

13.3 year Median Survival -- with RT + PCV

11

Ependymoma --
who ?

Gross path ?

buzzword for histo path?

more common peds tumors

Gross Path - well demarcated lesions

Histopath --
Pseudorossettes -- lack true lumen

Rossettes -- True Lumen

12

• Myxopapillary Ependymoma --

where does it occur?
presenting symptom?
benign vs malignant?

benign lesion of the filum terminale

Sx -- back pain

13

pituitary Adenomas --
How common?
where do they occur?
presenting symptoms related to this location?

what percentage are secretory?

10-15% of all CNS neoplasms

Arise in Sela Tursica
Can lead to diplopia/bitemporal hemianopia

70% are secretory

14

Treatment for prolactinoma?
Treatment for GH secreting tumor?

Prolactima -- Dopamine Agonists (Bromocriptin, Cabergoline)

GH secreting tumors -- Octreotide (somatostatin analog)

+ radiotherapy

15

Meningioma
- how common is it?
- from what cells do they originate? where do they arppear?

The most common benign CNS Primary (90% are benign)


- Arise from arachnoid cap cells

- commonly at the dural base and well circumscribed

16

Meningioma --
molecular pathology?
Histopathology?

-- Loss of 22q12.2 tumor suppressor (including Merlin/Schwannoma)

-- Histo: "Whorls" and Psammoma bodies

17

Meningioma Treatment?

what is the best predictor of failure/recurrence?

GTR

STR + External Beam Radiation

• Extent of the resection is predictor of failure

18

name 4 childhood primary tumors

Pilocytic Astrocytoma (benign, good prognosis)

medulloblastoma (Malignant)

Ependyoma (poor prognosis)

Craniopharyngioma (Benign)

19

Pilocytic Astrocytoma --
where does it commonly occur?

Gross path?

Histopath?

Posterior Fossae (Cerebellum)

Gross Path; Cystic appearance with "mural nodule"

Histo Path? Biphasic Architecture + rosenthal Fibers

20

medulloblastoma
classified as a ...?
where does it occur?
Gross Path?
Histopath?

Tendency for what other manifestation?

pathogenesis/progression?

Treatment?

Primitive Neuroendocrine Tumors (PNET), but occurs in the cerebellum and therefore Medulloblastoma

Gross Path -- Poorly circumscribed with necrotic hemorrhage

Histopath -- Homer Write Pseudorossettes

Tendency for CSF obsturction leading to hydrocephalus

Progression: disease is very aggressive and spreads through the CSF

Treatment: Surgical treatment followed by Craniospinal Radiation and Chemotherapy

21

Craniopharyngioma

- who gets these tumors?
- benign vs malignant?
- Arises from what embryological structure?
Treatment ?

Benign tumor of children, but there is bimodal distribution and adults can get them too

Arise from remnants of Rathke's Pouch

Treatment: GTR > STR + RT

22

Hemangioblastoma --
associated with what familial disease?

imaging findings?
where does it commonly present?

produces what hormone?

Histology?



A/w Von Hippel Lindau

imaging: Cystic Mural nodule in the cerebellum

Can produce EPO and lead to secondary polycythemia

Histo: Thin walled capillaries with minimal intervening parenchyma

23

what is the classic peripheral nerve sheath tumor?

8th cranial nerve schwannaoma

24

8th cranial nerve schwannoma

aka

- where is the lesion seen on imaging?

Histopath findings?

commonly seen in what tumor syndrome?


aka Vestibular schwannoma

Imaging: Cerebellopontine angle;

Histo:
Antoni A -- pink with "Verocay bodies" --- (the nuclear free zones)

Antoni B -- pale


Commonly seen with NF2


25

Treatment for Vestibular schwanomma ?

risk of the treatment

If unilateral -- Surgery
Risk - Deafness

Radiation

If Bilateral -- Bevacizumab;

26

Name 4 tumor syndromes ?

NF1 --

NF2 --

Tuberous Sclerosis

Von Hipple Lindau

27

NF1 --
genetic mutation?
Associated lesions ?
Pathognomonic histo?

Loss of Neurofibromin Gene (tumor suppressor gene)

lesions -- Cafe Au Lait, Lisch Nodules of the Iris, skin tumors, pheocromocytoma

Plexiform neurofibroma --

28

NeuroFibromatosis Type 2

- genetic mutation?
- Classic Tumor ?

merlin/schannomin (tumor suppressor loss)


Specific for Bilateral 8th nerve schwannomas

29

Tuberous Sclerosis --

genetic mutation?

CNS Lesions ?

Common manifestation?

Two genes which comprise a tumor suppressor complex; either can be mutated (chromosome 9 or chromosome 16)

CNS lesions --- cortical tubers; subependymal nodules, subependymal giant cell astrocytomas (obstructing outflow)

Present with Seizures

30

Von Hipple Lindae Disease

common tumor?
what other locations may tumors be outside the CNS

genetic mutation?

hemangioblastomas of the CNS

Renal cell carcinomas, Pheochromocytoma

○ Loss of tumor suppressor gene on chromosome 3p25.3 --- involved with HIF1 metabolism


31

chromosome(s) involved in Tuberous sclerosis ?

types of tumors found

common presentation

9 and 16

Facial tubers

cortical tubers

Subependylmal nodules

Subependymal giant cell astrocytoma (if it obstructs CSF flow) == pathognomoninc for this disease

sz

32

tumors which can present with NF2

bilateral 8th nerve schwannomas

Ependyomas, meningiomas