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NMSP > Tumors Dr. Martin > Flashcards

Tumors Dr. Martin Flashcards

1
Q

Most tumors in kids arise where

A

Posterior fossa : brainstem, cerebellum 4th ventricle, top of cord

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2
Q

Most common tumors are found where in adults

A

Cerebral hemispheres : above tentorium, or into temporal lobes

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3
Q

Most common adult tumor

A

Glioblastoma

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4
Q

Most common adult tumor

A

Embryonal = medulloblastoma , Pilocytic Astrocytoma

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5
Q

New onset headache or seizure what should you thing

A

Tumor

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6
Q 
GRADE 
1
2
3
4
A

1 : resect (Pilocytic astrocytoma)
2 : infiltration (recur), usually (diffuse astrocytoma or oligodendroglioma)
3 : radiation, chemo a, anaplastic astrocytoma
4 : Glioblastoma , usually fatal after 2years + NECROSIS, VEGF vascular proliferation

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7
Q

Glioblastoma usually comes from

A

Radiation therapy

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8
Q

Lower grades are for and higher grades are for

A

The lower the younger pts and the higher the older the pt

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9
Q

Infiltrating astrocytoma GRADE 2

  1. What type and location
  2. SX
  3. Stains
  4. Prognosis
A
  1. Cerebrum, primary astrocytoma
  2. Headache or seziures that’s new onset
  3. IDH1 R132H AND IDH1 + IDH2 if needed AND GFAP+ stain in astrocytes
  4. IDH Mutant has higher then IDH wild , usually over 5ys and then become higher grade
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10
Q

Diffuse Astrocytoma looks how

A

Glassy and side effect is bulging towards septum pellucidum

= loose suicide at location + loose grey/white junction

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11
Q

Astrocyte vs Oligodendrocyte shape

A 
Astrocyte = oval
Oligodendrocyte = round
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12
Q

Pilocytoma Astrocytoma GRADE 1

  1. Who and location
  2. Gene and predisposed by what
  3. Looks like
A
  1. 1st 2 decades in life cerebellum + 3rd ventricle
  2. IAA1549-BRAF gene, NF1 loss in neurofibromas predisposes
  3. Looks like : well -circumscribed, CYSTIC + MURAL NODULE(ball) =resect
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13
Q

Pilocytic astrocytoma

HISTOLOGY : cells seen and other characteristics

A
  1. Hair like cells with long bipolar processes
  2. Rosenthal Fibers (has time to form in this since its very slow)
  3. Eosinophilic granular bodies EGB
  4. Can extend into subarachnoid space only not aggressive and only can effect optic N
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14
Q

GFAP+ stain means what

A

ASTROCYTOMA any type

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15
Q 
Glioblastoma GRADE 4 
1. Gene mutation and causes what to be unregulated 
2. Looks like what 
3. Primary 
4 secondary
A
  1. CDKN2A deletion = activated EGFR or PDGFR, + resist apoptosis TP53
  2. Contrast ring enhancing lesion + central necrosis
  3. Older pt, no precursor lesion
  4. Younger pt, from low grade lesion TP53, IDH1 R132H mutation has better prognosis then IDH-wild
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16
Q

Glioblastoma IDH-wild mutations

A

TERT + EGFR

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17
Q

Glioblastoma 3 HISTOLOGICALLY Thigns SEEN = HALLMARK

A
  1. necrosis = serpentine looks like a line irregular form
  2. Pseudo-palisading : cells around necrosis lining vertically
  3. Vascular proliferation (looks like a glomerulus) = Due TO VEGF
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18
Q

Glioblastoma survival

A

25% alive after 2 years if wild

2-3years if mutant

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19
Q

When do you see ring enhancing lesion

A
  1. Glioblastoma

2. Abscess

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20
Q

Oligodendroglioma

  1. Who and location
  2. Sx
  3. Looks like
A
  1. 4th-5th decade, cerebral hemispheres PRIMARY in the WHITE MATTER
  2. Neuro problems like seizures for example for years
  3. Calcification in cortex along gyri
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21
Q

Oligodendroglioma histology

A
  1. Perineuronal satellitosis, (hug neurons)
  2. Perivascular aggregation (hug BVs)
  3. Perinuclear halos = FRIED EGGS
  4. delicate anastomosing capillaries = CHICKEN WIRE (BVs all over like honeycomb like wire fence)
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22
Q

Oligodendroglioma GRADE 2

  1. Gene
  2. Mutation
  3. Grade 3 is called what and histology
  4. Prognosis
A
  1. IDH1 and IDH2 mutation,
  2. 1p19q loss
  3. Anaplastic Oligodendroglioma: necrosis, vascularization
  4. 15-20yrs grade 2, 10-15yrs grade 3
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23
Q

Ependymoma GRADE 2 (can be grade 3)

  1. Who and location
  2. Gene
  3. Mutation
A
  1. 1st decade children (4th ventricle) + Adults (Spinal cord)
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24
Q

Ependymoma Histology

A
  1. Rosettes ** (TRUE ROSETTES + can see perivascular rosettes)
    = FLEXNER ROSSETTS are true
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25
Q

Ependymoma prognosis

A

Depends on how much is respected and how much is left behind

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26
Q

Mxyopapillary Ependymoma

A

GRADE 1

Film terminals location in papillary pattern in spine L2/L3= pain

27
Q

Subependymoma

A

GRADE 1
Lateral or 4th ventricle location
= causes obstructive hydrocephalus
= SEE ROSSETTES

28
Q

When working with mucin tumors thing about

A

Not bursting any cells or injuring them = material can leak out and cause reaction = can lead to fibrosis and other problems (during surgical removal)

29
Q

Choroid Plexus Papilloma

  1. Who
  2. Location
  3. Can look like what
  4. Causes what *
A
  1. Children (lateral ventricles) + Adults (4th ventricle)
  2. Choroid Plexus carcinoma which makes even more CSF always leading to obstruction
  3. A lot of CSF and hydrocephalus due to obstruction
30
Q

Colloid Cyst or 3rd Ventricle

  1. Who
  2. Location
  3. Causes what
  4. Prognosis and characteristic
A
  1. Young adults
  2. 3rd ventricle roof = Obstructs Foramen Monro*
  3. Noncommunicating hydrocephalus
  4. Fatal fast and can be positional (laydown induces obstruction)
31
Q

Ganglioglioma GRADE 1 (what are they)

  1. Sx
  2. Location
  3. Gene
A

Glioneural tumors

  1. Recurring seizures that don not respond to anti-epileptic drugs
  2. Temporal lobes, slow growing, surgically remove
  3. BRAF (V600E)
32
Q

Dysembryoplastic Neuroepithelial Tumor (DNET) GRADE 1

  1. Is what
  2. Sx
  3. Location and special FEATURE Seen
A
  1. Glioneuronal tumor
  2. Epilepsy not respond tot therapy, surgically remove
  3. Temporal lobes, MUCIN-RICH nodules floating around (look like Oligodendrocyte)
33
Q

EMBRYONAL Tumors

  1. Most common one
  2. 2 others
  3. Who and looks
A
  1. Medulloblastoma = posterior fossa
  2. Atypical Teratoid / Rhabdoid tumor (AT/RT) + Primitive Neuroectodermal tumor (PNET)
  3. Children, primitive and undifferentiated
34
Q

Medulloblastoma GRADE 4

4 types AND the genes and prognosis

A
  1. WNT : older kids, CHR6, B-Catenin,(90% survival in 5years = good)
  2. SHH : infants , young adults, MYCN amplification, (intermediate prognosis)
  3. Group 3 : MYC amp* + 17(I17Q)* infants children, CLASSIC + LARGE CELL (WORST PROGNOSIS)
  4. Group 4 : I17Q, NO MYC*, can have MYCN, has large cells, classic (BAD prognosis)
35
Q

Medulloblastoma GRADE 4

  1. Location and can cause what to happen
  2. Spreads where
  3. Tumor that usually causes what
A
  1. Cerebellum , midline = occluded CSF = hydrocephalus
  2. Subarachnoid space
  3. Drop Metz = disseminates through CSF down to Caudal equine
36
Q

Medulloblastoma GRADE 4

  1. Stain
  2. Histology
  3. tx
A
  1. Ki-67 proliferation marker
  2. Homer-Wright Rosettes + Cell wrapping (Cell in cell), high friability
  3. High radiosensitive
37
Q

Medulloblastoma GRADE 4

  1. Gene for classic and chromosome
  2. The types and what they are Associated with
A
  1. PTCH1, chr 9
    = PTCH1 : Nevoid BCC (NBCCS, Gorlin syndrome)
    = WNT : Familial Adenomatous Polyposis (APC)
    = SHH : LI-Fraumeni Syndrome (TP53)
38
Q

AT/ RT GRADE 4

  1. gene
  2. Location
  3. Stains
A
  1. CHR22, HSNF5 / INI1 **
  2. Posterior Fossa (cerebellum , like Medulloblastoma )
    • EMA** ,+ Vimentin**, -Desmin and - myoglobin
39
Q

Perivascular psudorosettes think

A

Ependymoma

40
Q

AT/ RT GRADE 4 prognosis and who

A

Less then 1year

Children under 3yo

41
Q

Primary CNS Lymphoma

  1. Who and type
  2. Stains
  3. What can trigger this
  4. Histology buzz word
A
  1. Older adults that are imunsuppresed AIDS or transplant , PRIMARY
  2. CD20 B-cell,
  3. EBV
  4. Hooping = cells separated by RETICULIN (silver stain)
42
Q

Germ Cell Tumor

  1. Location
  2. Who and ethnicity
  3. Most common one and its MARKERS
  4. TX
A
  1. Midline locations , Pineal Gland
  2. Male, Japanese and European, 1st 2 decades in life
  3. GERMINOMA ( AFP, B-HCG)
  4. Radiation very good response
43
Q

Pineal Gland tumors (3 of them, who, and gene and grade)

A
  1. Pineblastoma = HIGH grade , necrosis, KIDS, RB gene
  2. Pinecytoma = LOW grade, ADULTS
  3. Germ cell tumor like GERMINOMA = MOST COMMON and goes away after radiation
44
Q

Meningioma

  1. Grade
  2. How do they operate
  3. Gene and chr
  4. WHO *
A
  1. 1
  2. Grows even penetrating bone. Only not through brain = compresses brain
  3. NF2 (22q12) chr 22
  4. Radiation induced , 3rd decade
45
Q

Meningioma

  1. Looks like buzz words
  2. Stain
  3. Histology
A
  1. Bosselated (on dura) + en plaque
  2. EMA +
  3. Calcified Psammoma bodies + whorled clusters of monotonous cells
46
Q

Meningioma Atypical VS Anaplastic

A

ATYPICAL : GRADE 2 , more aggressive and recurring , clear and choroid
ANAPLASTIC : GRADE 3 , looks like carcinoma or sarcoma

47
Q

Psammoma bodies look like

A

Onions

48
Q

Meningiomas have what receptors and can grow extremely fast when

A
  1. Has progesterone receptors

2. During pregnancy then shrinks after delivery

49
Q

Metastatic tumors in brain

  1. Most common
  2. Common secondary tumors
  3. Tumor is causing hemorrhage
  4. Location
A
  1. Metastatic carcinoma
  2. Lung, breasts, melanoma kidney , Choriocarcinoma (usually), prostate (rarely)
  3. Melanoma, choriocarcinoma, Renal Cell Carcinoma, Lung
  4. Meninges usually at grey/white junction
50
Q

Prostate cancer likes going

A

Through nerves so to SPINE

51
Q

Paraneoplastic Syndromes ** BOARDS**

  1. Subacute cerebellar Degeneration
  2. Limbic Encephalitis
  3. Eye movement disorder
  4. Subacute sensory neuropathy
  5. Lambert - Eaton Myasthenic Syndrome
A
  1. PCA-1 AB destruction of Purkinje cells (Ovarian, uterine, breast CARCINOMA)
  2. Subacute dementia,
    = ANNA-1 AB : Small Cell Carcinoma,,,,
    = NMDA Receptor in hippocampus : Ovarian teratomas,,,,
    = VGKC AB : voltage-g K+ Channels Peripheral neuropathy
  3. Neuroblastoma
  4. Associated with limbic encephalitits
  5. Voltage gated CA+2 channels + malignancy
52
Q

4 syndromes associated with MEDULLOBLASTOMA and the genes involved

A
  1. Nevoid BCC = PTCH
  2. LI Fraumeni syndrome = TP53
  3. Turcot syndrome : also Glioblastoma , ACP
  4. Gorlin syndrome : PTCH, increases SHH
53
Q

Cowden syndrome is associated with what brain tumor and gene

A

PTEN ——> IK3 / AKT

Dysplastic Gangliocytoma

54
Q

Tuberous Sclerosis Complex

  1. Inheritance AND sx
  2. Genes involved and there proteins
  3. What tumors do you get and looks like what in brain
A
  1. AD, seizures, Autism, intellectual disability
  2. TSC1 = Hamartin, TSC2 = Tuberin
  3. Hamartomas, Candle Dutterings (was like dripping masses) = cortical tubur (oncortex mushroom shape)
55
Q

Tuberous Sclerosis Complex OTHER tumors you see with this

A
  1. Retinal Glial hamartomas **
  2. Renal Angiomyolipomas
  3. Pulmonary Lymphangioleiomyomatosis
  4. Cardiac Rhabdomyomas
  5. Cutaneous lesions (Angiofibromas, subungal = shagreen patches, ash-leaf patches)
56
Q

Von Hippel Lindau Disese BOARDS

  1. Inherited, gene and chr
  2. Regulates what signaling
  3. Causes what 4 things
  4. Associated with what
A
  1. AD, VHL CHR 3p25.3
  2. HIF-1 and VEGF regulation
  3. = Polycythemia (high RBCs)
    = Hemangiomas in CNS (cerebellum and retina)
    = CYSTS in LIVER, PANCREAS, KIDNEY
    = RCC
  4. Phenochromocytoma
57
Q

NF1

  1. Gene and chr, and protein made
  2. What is causes
  3. PE findings
A
  1. NF1 gene, 17q11.2 = neurofibromas
  2. = Neurofibromas
    = Optic nerve Gliomas
    = Lisch nodules (iris pigment nodules)
  3. Cafe au lait spots
    (MOST COMMON TYPE)
58
Q

NF2

  1. Gene and chr, and protein made
  2. What is causes
  3. PE findings
A
  1. Gene NF2, 22q12 = Merlin
  2. Bilateral Schwannomas CN8 **
    = many meningiomas + ependymomas of Cervical spine
59
Q

Schwannomatosis

A

Many nonvestibular Schwannomas throughout body or in one place (Around nerves **, the Neurofibromas are INSIDE NERVE and cant get respected off)

60
Q

3 peripheral nerve sheath tumors

A

1 Schwannomas
2, Neurofibromas
3 MPNST

= Schwann cell differentiation tumors

61
Q

Schwannomas

  1. Gene and STAINS and protein loss
  2. What happens
  3. Location
  4. 2 types
A
  1. NF2, S100+, loss of Merlin
  2. Caustic neuroma CN8 (tinnitus and hearing loss)
  3. Cerebellar pontine angle
  4. = Antoni A DENSE : VEROCAY BODIES (palisading nuclei around nuclear free zones)**
    = Antoni B LOOSE : hypocellular myxoid ECM
62
Q

PLEXIFORM neurofibromas looks like what

A

Bag of worms (NF1)

63
Q

MPNST

  1. Gene and type of tumor
  2. Grade
A
  1. NF1 , usually comes from NF1 , a subtype is Triton tumor , rhabdomyoblastic
  2. HIGH Grade
64
Q

When do you see Homer Wright Rosettes and when do you see pseudorosettes and true rosettes

A

Homer Wright Rosettes : Medulloblastoma

Pseudorosettes + True rosettes = Ependymoma

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