ALL CNS TUMORS - Random order!!!! Flashcards Preview

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Flashcards in ALL CNS TUMORS - Random order!!!! Deck (42)
1

Tumors found in kids?

ependyoma

Pilocytic Astrocytoma

Medulloblastoma

Craniopharyngeoma

2

What is a central Neurocytoma?

Intravenctricular --> LATERAL VENTRICLE tumor with neuronal differentiation

 

see small benign looking cells that stain with Synaptophysin 

2

von Hippel Lindau presentation 

Hemangioblastomas of CNS!!!!

Renal Cell CArcinoma

Pheochromocytomas 

Microcytic Adenomas of Pancreas!!!! 

 

If you see hemangioblastoma in younger patients, check kidneys and adrenals 

3

Presentation of Oligodendrogliomas and appearance? 

Typically adult patients and likes to grow in frontal lobes and White Matter and can cause seizures

 

Grows in White Matter, Calcifications!

 

See: Rounded cell contours with perinuclear halos = Fried Eggs

See: Plexiform background network = Chicken wire

3

What is a Craniopharyngioma? PResentation and cytology? 

 

Treatment?

More common in children but Bimodal Distribution 

Benign tumor from remnants of Rathke's Pouch with both Cystic and Solid portions = Pharyngeal invaginations 

 

Tx: Surgery Trans-sphenoid gives higher GTR

Radiation therapy after sub-total resection 

4

What the HECK is a Dysembryopastic Neuroepithelial Tumor (DNT)?

Mixed Neuronal and Glial Tumor

 

Benign

Commonly presents in younger patients with Long history of partial complex seizures

 

*Young ppl with epilepsy --> Check Temporal lobe

5

What are the features of a Medulloblastoma? How is it staged? Treatment?

See granular cells of the cerebellum - smal, round, blue cells

 

Homor Write Pseudorosette with fibrillary processes into the lumen 

Grade 4 tumor that can be cured

Staging is either "average" or "high risk" 

Treatment for both stages is Resection and External bean radiation therapy 

can also add chemotherapy 

5

What are the different types of pituitary adenomas?

Prolactinomas - most common

GH-producing adenomas (Somatotrophs) 

ACTH- producing adenomas (somatotrophs) 

TSH-producing adenomas (thyrotrophs) 

5

Treatment for Prolactinoma?

Bromocriptine and Cabergoline = Da Agonists and radiation therapy

6

What is a grade 1 astrocytome? who gets it? what you do see? 

Pilocytic Astrocytoma - not diffuse!

Benign tumor in children and young adults that presents in the Cerebellum 

 

Cysts with Mural Nodule

Rosenthal Fibers - protenacious accumulations of intermediate filaments in astrocites (thick eosinophilic processes, GFAP+) 

Biphasic PAttern - loose textured microcytic material next to dense eosinophilic material with hair-like processes

 

7

What are the different treatment options for meningioma?

Observation if asymptomatic

Surgery - GTR if possible 

+ Post-op RT for progression free survival if sub-total resection

8

What are the gross and microscopic findings in Glioblastoma Multiforms?

Circumscribed appearance (from necrosis and hemorrhage) that is an ENHANCING tumor on MRI with surrounding edema (mass effect) 

 

Glioblastoma with Butteryfly Pattern crossing the Corpis Collosum 

Vascular Proliferation and Necrosis with Pseudopalisading 

9

What are the microscopic indications for Meningioma? 

 

What are the genes? 

see Whorls and Psammoma Bodies 

 

Loss of 22q12 gene for Merlin/Schwannomin Tumor Suprpresor 

9

Von Hippel Lindau Disease inheritance and genetics?

Autosomal Dominant loss of tumor suppressor gene on Chromsome 3p25.3 (involved in HIF1alpha metabolism) 

10

What is the most common source for mets to the brain? Where do Mets go? what do they look like? 

Lung tumors 

Mets are usually multiple and live at the Gray-White Junction 

see Circumscirbed lesions with Vasogenic Edema 

10

What are treatments for Brain Mets?

Whole Brain RT + resection if single mets or need emergent decompression

 

[Chemotherapy limited bc poor BBB permeability]

10

Treatment for ACTH producing tumors? 

Mitotane, Ketoconazole, Metapyrone

 

RAdiation therapy 

11

NF2 inheritance and genes, presentation?

Autosomal Dominant loss of chromosome 22q12.2 Merlin/Schwannomin tumor suppressor gene

 

Bilateral CN 8 Schannomas (tx Bevacizumab) 

Also associated with Ependymomas and meningiomans 

13

What is a myxopapillary ependyoma? who gets it? how does it present?

Grade 1 Ependyoma

 

Benign and typically seen in young adults in the Filum Terminale 

*Back Pain in young adults

TX: Surgery curative 

 

Can see ECM and ground substance in the tumor 

 

 

 

15

What is the genetic mutation in pilocytic astrocytoma?

7q34 Duplication producing BRAF fusion

 

B.R.A.F = 

Biphasic Pattern

Rosenthal Fibers

All kids are going to be 

Fine!

16

What is the mutation sequence for gliomas?

First TP53 mutation

Then IDH-1 Mutation which is low grade 2/3 tumors of astrocytes or oligos and indicates favorable prognosis and marker for late benefit from chemotherapy 

1p/19q = Oligodendrogliomas (predictive and prognostic marker) 

vs 

ATRX mutation = Astrocytomas 

17

Primary vs Secondary Glioblastoma?

Primary (de novo) = Older patients and increased EGFR gene amplifications; tend to be Grade 4 at presentation 

 

Secondary = younger patients and have progression from low grade to high grade (TP53 --> IDH1) 

18

What is a Schwannoma? Presentaiton? 

Benign tumor of vestibular nerve sheath 

*Unilateral hearing loss/ringing/tinnitis  in adults!!! 

Grow eccentrically around CN8 - acoustic schwannoma or neuroma 

 

If Bilateral = Think NF2!!! 

20

PRognostic/Predictive variable in Glioblastoma?

MGMT Methylation is Prognostic - patients do poorer if unmethylated no matter treatment

and Predictive - patients do better if methylated with treatment of Temozolomide 

21

What is a Hemangioblastoma? Who gets it? 

Where does it occur?  what does it look like?

Adult tumor in Cerebellum (*May present with polycythemia - from EPO production)

either sporadic or familial with Von-Hippel Lindau

 

see Cyst with mural nodule in cerebellum

 

Lipidized stromal cells with Foamy Cytoplasm 

23

What are the treatments for Glioblastoma? What genetics are predictive?

Methylation of MGMT = convers improved progression free survival in pts with radiation and Temozolomide (alkylating agent) 

-If tumor has methylated MGMT then it can't function properly to remove DNA damage from Temozolomide and it's more effective 

 

GTR almost impossible so Tx with Radiation + Temozolomide 

 

New Therapy Novo TTFields - low amplitude electric currents disrupts interface with cell division (similar to microtubule poison) 

24

Genetics of Tuberous Sclerosis 

Autosomal Dominant mutations in TSC1 (Hamartin) or TSC2 (tuberin) 

 

Chromosomes 9 and 16

25

NF1 genetics, Inheritance and presentation?

Autosomal Dominant loss of chromosome 17q11.1 (neurofibromin down regulates p21ras) 

 

See Plexiform Neurofibroma - tumor that expands in the nerve in sheets and fascicles

 

Associated LEsions: cafe au lait spots, Pheochromocytoma, gliomas of optic nerve and hypothalamus 

26

Differential Dx for blob tumor in ventricle?

Ependymoma

Choroid Plexus Tumor

Central Neurocytoma 

27

What are the treatment options for schwannoma? 

Observation if growing/shrinking

Surgery but preservation of facial nerve and hearing is difficult

 

Radiosurgery of tumors

Bevacizumab used for bilateral tumors to reduce size and preserve hearing 

28

PRognostic and Predictive Markers for Oligodendrogliomas?

1p/19q Codeletion  = Prognostic Marker (improved progression free survival) 

= Predictive Marker (chemosensitivity - esp grade 3 tumors)

Codeletion is a molecular marker and you do better if you have the deletion no matter what!!! 

30

What is a Meningioma? Who gets it? Where does it occur?

Meningioma is the most common benign primary brain tumor in adults

Grade 1 are majority and Grade 2 or 3 are rare

Adults get it - incidence increases with Age

FEMALES > Males (hormone receptors on tumor to E/P - can grow in pregnancy)

seen in the dural attachment but derived from meningothelial cells in arachnoid "arachnoid cap cells" 

Well-circumscribed mass in dura

32

What are the tumors of the choroid plexus?

Choroid plexus papilloma = young patients, low grade benign; Intraventricular

 

Choroid plexus Carcinoma = higher grade, can be seen in infancy

33

Presentation of Tuberous Sclerosis 

Skin lesions, Seizures, Mental REtardation 

 

Skin findings ( adenoma, sebaceous, macules and patches etc) 

Renal Angiomyolipomas, Pulmonary lymphangiomyomatosis and cardiac rhabdomyomas 

aka Kindey, Lyng, and Heart tumors

See Subependymal Nodules = Candle Wax

Cortical Tubers = potato cortex that causes seizuers 

35

What is an Ependymoma? What is it's presentation? What do you see in it? Genetics?

Tumor of Ependymal cells --> Grade 2 or 3

Presentation: KIDS in 4th ventricle and get hydrocephalus or Adults in Spinal Cord

*Deletion of Chromosome 22

 

See: True Ependymal rosettes (true lumen) and Perivascular Pseudorosettes

36

Tumors in the Cerebellum

PCA

Medulloblastoma

Hemangioblastoma 

37

What is a medulloblastoma? Who gets it? Where does it occur? 

Primitive Neuroectodermal Tumor (PNET) of the Cerebellum = Neuroectoderm Granular cells of the cerebellum turn into a tumor 

Malignant tumor in Kids (men>women, Whites>blacks) 

Cerebellum and can seed from 4th ventricle  to spine = Drop Mets wihch are bad!!!! 

38

Treatment for GH-producing ademonas?

Somatostatin analogs like Ocreotide and Lanreotide 

Pregvisomant - IGF inhibiotr $$$$

 

Radiation therapy!!! 

39

Who gets pituitary ademonas? How do they present?

Females > MAles ages 45-55

Majority are benign; occur midline in the Sella Turcica 

Diplopia is the most common presentation bc they press on the optic chiasm 

ALL are anterior pituitary - no posterior lobe tumors 

 

Can be Microadenomas Macroademoas >10mm or Giant Adenomas >40mm

40

What is the cytology of a Schwannoma? What are the genetics?

Antoni A with Verocay Bodies  (pink with palisading organization of cells) and Antoni B (pale staining) 

 

If bilateral, think NF2 inactivating mutations of Tumor Sppressor gene on 22q12 = MERLIN protein 

41

What tumors present with Cysts with Mural Nodules?

PCA - kids in cerebellum; chrom 7 duplication BRAF, Biphasic, rosenthal fibers, benign

 

Hemangioblastoma - adults sporadic or Von Hippel, lipiized stromal cells with Foamy cytoplasm, increase EPO - polycythemia, 

42

What is the difference between a Grade 2 and Grade 3 Astrocytoma?

 

What is the difference between a Grade 3 and Grade 4?

Grade 2 =  can be treated with Maximum Safe resection and Adjuvant ChemoRT to prolong progression free survival 

Grade 3 = Increased Mitotic Activity!!!! Increased cellularity and pleomorphism 

 

Grade 4 = Vascular Proliferation and Necrosis - see necrosis and pseudopalisading areas; enhancing tumor on MRI with surrounding edema (mass effect)