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PT2T1 > ALL CNS TUMORS - Random order!!!! > Flashcards

ALL CNS TUMORS - Random order!!!! Flashcards

1
Q

Tumors found in kids?

A

ependyoma

Pilocytic Astrocytoma

Medulloblastoma

Craniopharyngeoma

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2
Q

What is a central Neurocytoma?

A

Intravenctricular –> LATERAL VENTRICLE tumor with neuronal differentiation

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

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2
Q

von Hippel Lindau presentation

A

Hemangioblastomas of CNS!!!!

Renal Cell CArcinoma

**Pheochromocytomas **

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

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

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3
Q

Presentation of Oligodendrogliomas and appearance?

A

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

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3
Q

What is a Craniopharyngioma? PResentation and cytology?

Treatment?

A

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

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4
Q

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

A

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

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5
Q

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

A

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

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5
Q

What are the different types of pituitary adenomas?

A

Prolactinomas - most common

GH-producing adenomas (Somatotrophs)

ACTH- producing adenomas (somatotrophs)

TSH-producing adenomas (thyrotrophs)

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5
Q

Treatment for Prolactinoma?

A

Bromocriptine and Cabergoline = Da Agonists and radiation therapy

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6
Q

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

A

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

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7
Q

What are the different treatment options for meningioma?

A

Observation if asymptomatic

Surgery - GTR if possible

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

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8
Q

What are the gross and microscopic findings in Glioblastoma Multiforms?

A

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 **

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9
Q

What are the microscopic indications for Meningioma?

What are the genes?

A

see Whorls and Psammoma Bodies

Loss of 22q12 gene for Merlin/Schwannomin Tumor Suprpresor

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9
Q

Von Hippel Lindau Disease inheritance and genetics?

A

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

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10
Q

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

A

Lung tumors

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

see Circumscirbed lesions with Vasogenic Edema

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10
Q

What are treatments for Brain Mets?

A

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

[Chemotherapy limited bc poor BBB permeability]

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10
Q

Treatment for ACTH producing tumors?

A

Mitotane, Ketoconazole, Metapyrone

RAdiation therapy

11
Q

NF2 inheritance and genes, presentation?

A

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
Q

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

A

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
Q

What is the genetic mutation in pilocytic astrocytoma?

A

7q34 Duplication producing BRAF fusion

B.R.A.F =

Biphasic Pattern

Rosenthal Fibers

All kids are going to be

Fine!

16
Q

What is the mutation sequence for gliomas?

A

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
Q

Primary vs Secondary Glioblastoma?

A

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
Q

What is a Schwannoma? Presentaiton?

A

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
Q

PRognostic/Predictive variable in Glioblastoma?

A

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

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

21
Q

What is a Hemangioblastoma? Who gets it?

Where does it occur? what does it look like?

A

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
Q

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

A

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
Q

Genetics of Tuberous Sclerosis

A

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

Chromosomes 9 and 16

25
Q

NF1 genetics, Inheritance and presentation?

A

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
Q

Differential Dx for blob tumor in ventricle?

A

Ependymoma

Choroid Plexus Tumor

Central Neurocytoma

27
Q

What are the treatment options for schwannoma?

A

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
Q

PRognostic and Predictive Markers for Oligodendrogliomas?

A

**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
Q

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

A

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
Q

What are the tumors of the choroid plexus?

A

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

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

33
Q

Presentation of Tuberous Sclerosis

A

**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
Q

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

A

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
Q

Tumors in the Cerebellum

A

PCA

Medulloblastoma

Hemangioblastoma

37
Q

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

A

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
Q

Treatment for GH-producing ademonas?

A

Somatostatin analogs like Ocreotide and Lanreotide

Pregvisomant - IGF inhibiotr $$$$

Radiation therapy!!!

39
Q

Who gets pituitary ademonas? How do they present?

A

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
Q

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

A

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
Q

What tumors present with Cysts with Mural Nodules?

A

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
Q

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

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

A

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)

PT2T1 (38 decks)

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