ALL CNS TUMORS - Random order!!!!

Question 1

Tumors found in kids?

Answer 1

ependyoma

Pilocytic Astrocytoma

Medulloblastoma

Craniopharyngeoma

Question 2

What is a central Neurocytoma?

Answer 2

Intravenctricular –> LATERAL VENTRICLE tumor with neuronal differentiation

see small benign looking cells that stain with **Synaptophysin **

Question 2

von Hippel Lindau presentation

Answer 2

Hemangioblastomas of CNS!!!!

Renal Cell CArcinoma

**Pheochromocytomas **

**Microcytic Adenomas of Pancreas!!!! **

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

Question 3

Presentation of Oligodendrogliomas and appearance?

Answer 3

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

Question 3

What is a Craniopharyngioma? PResentation and cytology?

Treatment?

Answer 3

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

Question 4

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

Answer 4

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

Question 5

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

Answer 5

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”

T**reatment for both stages is Resection and External bean radiation therapy **

can also add chemotherapy

Question 5

What are the different types of pituitary adenomas?

Answer 5

Prolactinomas - most common

GH-producing adenomas (Somatotrophs)

ACTH- producing adenomas (somatotrophs)

TSH-producing adenomas (thyrotrophs)

Question 5

Treatment for Prolactinoma?

Answer 5

Bromocriptine and Cabergoline = Da Agonists and radiation therapy

Question 6

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

Answer 6

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

Question 7

What are the different treatment options for meningioma?

Answer 7

Observation if asymptomatic

Surgery - GTR if possible

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

Question 8

What are the gross and microscopic findings in Glioblastoma Multiforms?

Answer 8

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 **

Question 9

What are the microscopic indications for Meningioma?

What are the genes?

Answer 9

see Whorls and Psammoma Bodies

Loss of 22q12 gene for Merlin/Schwannomin Tumor Suprpresor

Question 9

Von Hippel Lindau Disease inheritance and genetics?

Answer 9

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

Question 10

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

Answer 10

Lung tumors

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

see Circumscirbed lesions with Vasogenic Edema

Question 10

What are treatments for Brain Mets?

Answer 10

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

[Chemotherapy limited bc poor BBB permeability]

Question 10

Treatment for ACTH producing tumors?

Answer 10

Mitotane, Ketoconazole, Metapyrone

RAdiation therapy

Question 11

NF2 inheritance and genes, presentation?

Answer 11

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

**Bilateral CN 8 Schannomas (tx Bevacizumab) **

Also associated with Ependymomas and meningiomans

Question 13

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

Answer 13

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

Question 15

What is the genetic mutation in pilocytic astrocytoma?

Answer 15

7q34 Duplication producing BRAF fusion

B.R.A.F =

Biphasic Pattern

Rosenthal Fibers

All kids are going to be

Fine!

Question 16

What is the mutation sequence for gliomas?

Answer 16

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

Question 17

Primary vs Secondary Glioblastoma?

Answer 17

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)

Question 18

What is a Schwannoma? Presentaiton?

Answer 18

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!!!

Question 20

PRognostic/Predictive variable in Glioblastoma?

Answer 20

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

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

Question 21

What is a Hemangioblastoma? Who gets it? Where does it occur? what does it look like?

Answer 21

**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

Question 23

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

Answer 23

**_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)

Question 24

Genetics of Tuberous Sclerosis

Answer 24

Autosomal Dominant mutations in TSC1 (Hamartin) or TSC2 (tuberin) Chromosomes 9 and 16

Question 25

NF1 genetics, Inheritance and presentation?

Answer 25

**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

Question 26

Differential Dx for blob tumor in ventricle?

Answer 26

Ependymoma Choroid Plexus Tumor Central Neurocytoma

Question 27

What are the treatment options for schwannoma?

Answer 27

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

Question 28

PRognostic and Predictive Markers for Oligodendrogliomas?

Answer 28

**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!!!

Question 30

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

Answer 30

*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 attachmen**t but derived from meningothelial cells in arachnoid **"arachnoid cap cells" ** _Well-circumscribed mass in dura_

Question 32

What are the tumors of the choroid plexus?

Answer 32

Choroid plexus papilloma = young patients, low grade benign; Intraventricular Choroid plexus Carcinoma = higher grade, can be seen in infancy

Question 33

Presentation of Tuberous Sclerosis

Answer 33

***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

Question 35

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

Answer 35

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_**

Question 36

Tumors in the Cerebellum

Answer 36

PCA Medulloblastoma Hemangioblastoma

Question 37

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

Answer 37

Primitive **Neuroectoderma**l 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!!!!

Question 38

Treatment for GH-producing ademonas?

Answer 38

Somatostatin analogs like Ocreotide and Lanreotide Pregvisomant - IGF inhibiotr $$$$ Radiation therapy!!!

Question 39

Who gets pituitary ademonas? How do they present?

Answer 39

**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

Question 40

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

Answer 40

**Antoni A with Verocay Bodies** (pink with palisading organization of cells) and **Antoni B** (pale staining) If bilateral, think NF2 inactivating mutations of T**umor Sppressor gene on 22q12 = MERLIN** protein

Question 41

What tumors present with Cysts with Mural Nodules?

Answer 41

**_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,

Question 42

What is the difference between a Grade 2 and Grade 3 Astrocytoma? What is the difference between a Grade 3 and Grade 4?

Answer 42

Grade 2 = can be treated with M**aximum 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)