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STEP 5: Neuro PATHOMA > CNS Tumors > Flashcards

CNS Tumors Flashcards

1
Q

Are CNS tumors more often metastatic or primary?

A

50% metastatic (from LUNG, BREAST, KIDNEY)

50% primary

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2
Q

Children primary tumors are most often ___?

Adult primary tumors are most often __? Supratentorial or Infratentorial?

A

CHILDREN: INFRATENTORIAL
ADULT: SUPRATENTORIAL
Children are shorter and so they’ll be infratentorial

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3
Q

CHILDREN: What is the most common PRIMARY CNS tumor What is the most common BENIGN PRIMARY CNS tumor? What are the two possible MALIGNANT PRIMARY CNS tumors?

A
  1. MOST COMMON = PILOCYTIC ASTROCYTOMA (benign tumor of astrocytes)
  2. MEDULLOBLASTOMA = Malignant tumor of granular cells of cerebellum (Neuroectoderm)
  3. EPENDYMOMA = Malignant tumor of ependymal cells
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4
Q

ADULT: What is the most common PRIMARY CNS tumor? What primary tumors are most common in adults? (1 malignant, 1 benign)

A

MOST COMMON = GLIOBLASTOMA MULTIFORME = MALIGNANT CNS TUMOR of ASTROCYTES

MENINGIOMA = Benign tumor of arachnoid cells in FEMALES
OLIGODENDROGLIOMA = Malignant tumor of oligodendrocytes
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5
Q

MNEMONIC for children and adult PRIMARY CNS TUMORS (MALIGNANT)

A

Children (immature) say ME ME! I want it - MEDULLOBLASTOMA + EPENDYMOMA
Adult (mature) know what they don’t want say GO (away cns tumor)! - GLIOBLASTOMA MULTIFORME + OLIGODENDROGLIOMA

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6
Q

What is the classic morphological change on BIOPSY of a GLIOBLASTOMA MULTIFORME (Adult malignant tumor of glial cell - astrocytes)?

A

BUTTERFLY LESION - Initially arises from the CEREBRAL HEMISPHERE -> Crosses the corpus luteum

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7
Q

What are the 2 histological changes of a GLIOBLASTOMA MULTIFORME?
What is the diagnostic test marker of this CNS tumor?

A
  1. PSUEDOPALISADING NECROSIS - Tumor cells lining around areas of necrosis [“BLAST off in a palisading manner”]
  2. ENDOTHELIAL MICROVASCULAR PROLIFERATION

GFAP+ = Marker = Intermediate filament of GLIAL CELLS

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8
Q

What is a MENINGIOMA? Who is the typical pt affected? What is the marker of a MENINGIOMA?

A

BENIGN tumor of ARACHNOID CELLS - Express estrogen receptor***
Typical pt = WOMEN ADULTS

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9
Q

What is the most common Sx of a MENINGIOMA?

A

SEIZURE

Tumor is attached to the dura -> Compresses the cortex but does NOT invade the cortex -> SEIZURES

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10
Q

What are two histological evidences of a MENINGIOMA?

A

“MEN (meningioma) and FEMALES (typical pt affected) like to WHORL around and CALCIFY together”

  1. WHORLED - spindled cells arranged in a whorled pattern
  2. PSAMMOMA BODIES - calcifications of the spindled cells
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11
Q

What is seen on imaging of a MENINGIOMA?

A

DURAL TAIL

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12
Q

What is a BENIGN TUMOR of SCHWANN CELLS (cells that myelinate PNS)? What is the most common nerve affected?

A

SCHWANNOMA - Can affect BOTH CRANIAL + SPINAL NERVES

CN VIII = most common nerve compressed by tumor as it exits at the CERBELLOPONTINE ANGLE

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13
Q

How does a SCHWANNOMA most commonly present clinically?

A

Most commonly involves CN VIII - TINNITUS, LOSS OF HEARING

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14
Q

What is the diagnostic marker of a SCHWANNOMA? What other pathologies also show this marker?

A

S100+

Also seen in LANGERHANS CELL HISTIOCYTOSIS + MELANOMA

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15
Q

What is associated with BILATERAL SCHWANNOMAS?

A

NEUROFIBROMATOSIS TYPE 2

Almost 100% of Schwannomas have bi-allelic inactivation of NF-Type II

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16
Q

What are 2 histologic features of SCHWANNOMAS?

A
  1. SPINDLE SHAPED CELLS with rod-like nuclei (S = spindle= schwannomas)
  2. VEROCAY BODIES = ALTERNATING hyocellular areas (antoni B) and hypercellular areas (antoni A)
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17
Q

What is the second most common MALIGNANT tumor in adults?

A

OLIGODENDROLGIOMA - Malignant tumor of oligodendrocytes (GO)

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18
Q

Where does an OLIGODENDROGLIOMA most commonly present in an adult? Thus, what sx is most likely?

A

MALIGNANT TUMOR OF oligodendrocytes = CALCIFIED tumor in WHITE MATTER of FRONTAL LOBE
Usually presents with SEIZURES**

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19
Q

What are the two histological features of an adult OLIGODENDROGLIOMA?

A

O = SHAPE OF AN EGG! Eggs come from a CHICKEN

  1. FRIED EGG APPEARANCE - Round nuclei with clear appearance
  2. CHICKEN WIRE CAPILLARY pattern - Nuclei are NOT lined up together
20
Q

What is a PILOCYTIC ASTROCYTOMA? Who is the typical pt? What is the most commonly involved structure?

A

MOST COMMON, BENIGN TUMOR of ASTROCYTES in the CEREBELLUM - Children pt
(cerebellum since most CNS Tumors are INFRATENTORIAL)

21
Q

What is seen on imaging of a PILOCYTIC ASTROCYTOMA?

A

PILOCYTIC, piloCYSTic

CYST with mural thrombus in the cerebellum

22
Q

What are 2 histological evidences of a PILOCYTIC ASTROCYTOMA?

A
  1. Elongated cells (eosinophilic granular bodies) with PILOID, hair like FIBERS
    Think PILO, LILLY, ROSE! - flowers
  2. ROSENTHAL, eosinophilic corkscrew FIBERS coming off of tumor cells
23
Q

What is the diagnostic marker of a PILOCYTIC ASTROCYTOMA?

A

PILOCYTIC ASTROCYTOMA = Tumor of astrocytes** of cerebellum = glial cell

GFAP+ = IMF of glial cells

24
Q

What is a MEDULLOBLASTOMA? Who is the typical pt? What is most commonly affected?

A

MALIGNANT TUMOR of NEUROECTODERMAL cells of the CEREBELLUM (infratentorium) of CHILDREN
“ME”

25
Q

ROSETTE FIBERS
HOMER WRIGHT ROSETTES
PERIVASCULAR ROSETTES

A

ALL CHILDREN PRIMARY CNS TUMORS have “roses” (Children like to pick up roses from the garden)

  1. Rosette fibers = Elongated fibers of a PILOCYTIC ASTROCYTOMA (Benign since it’s not “ME”)
  2. Homer wright rosettes = Blue cells surrounding neuritic processes of a MEDULLOBLASTOMA (Malignant)
  3. Perivascular rosettes = Ependymomas extend their processes AWAY from BV = Clearing from the BV= EPENDYMOMA
26
Q

What are 2 histological evidences of a MEDULLOBLASTOMA?

A
  1. MedulloBLastoma = BLue cells (small round, neoplastic blue cells)
  2. HOMER-WRIGHT ROSETTES = Blue cells surrounding pink neuritic processes
27
Q

Why does a MEDULLOBLASTOMA have a poor prognosis? Think in terms of metastasis.

A

Medulloblastoma can grow RAPIDLY into the 4th ventricle -> Spread into the CSF -> Spinal cord -> Metastasis to the cauda equina that extends into S2 = DROP METASTATIS

28
Q

What is DROP METASTASIS in relation to children primary CNS tumors?

A

MEDULLOBLASTOMA that arises in cerebellum -> Extends into 4th ventricle -> CSF -> spinal cord -> cauda equina

29
Q

What is an EPENDYMOMA? Who is the typical pt? Where does it most commonly affect?

A 
EPENDYMOMA = Malignant (ME) CNS tumor of EPENDYMAL CELLS (lining ventricles) in a CHILD 
4th ventricle (INFRATENTORIAL) = most commonly involved
30
Q

How does a child with EPENDYMOMA clinically present? What is the histological finding?

A

HYDROCEPHALUS: Due to tumor of ependymal cells lining 4th ventricle and extending into that space

PERIVASCULAR ROSETTES: Ependymal neuritic processes extended AWAY from BV = clearing from the BV

31
Q

Ddx of a BITEMPORAL HEMIANOPSIA in a child:

A

PITUITARY ADENOMA

CRANIOPHARYNGIOMA

32
Q

What is a CRANIOPHARYNGIOMA? Who is the typical pt? Where does it typically affect?

A

CRANIOPHARYNGIOMA = BENIGN Tumor arising from EPITHELIAL REMNANTS OF RATHKE POUCH = SUPRATENTORIAL MASS in CHILD or YOUNG ADULT

Normally: Rathke pouch loses connection with the upper protrusion of the pharynx (floor of the mouth) to form the ANTERIOR PITUITARY and sits on the sella turcica

33
Q

How does a CRANIOPHARYNGIOMA result in BITEMPORAL HEMIANOPSIA?

A

CRANIOPHARYNGIOMA - tumor of epithelial remnants of rathek pouch on sella turcica -> Can compress on the OPTIC CHIASM -> Bitemporal hemianopsia

34
Q

What is the classic imaging seen of a CRANIOPHARYNGIOMA?

A

Craniopharygioma - See CALCIFICATIONS, derived from “tooth-like” tissue

35
Q

Is a CRANIOPHARYNGIOMA typically malignant or benign? Can it be treated by resection

A

BENIGN but tends to recur after resection

36
Q

CALCIFICATIONS: WHORLED BODIES/PSAMMOMA
CALCIFICATIONS: CALCIFICATIONS of a SUPRATENTORIAL MASS

A

PSAMMOMA BODIES: Meningioma (malignant)

Calcifications of supratentorial mass: Craniopharyngioma (benign)

37
Q

ROSENTHAL FIBERS

EOSINOPHILIC GRANULAR FIBERS

A

PILOCYTIC ASTROCYSTOMA

38
Q

PERIVASCULAR ROSETTES

A

EPENDYMOMA

39
Q

HOMER-WRIGHT ROSETTES + SMALL BLUE CELLS

A

MEDULLOBLASTOMA

40
Q

BUTTERFLY LESION (Crossing over corpus callosum) + PSEUDOPALISADING

A

GLIOBLASTOMA MULTIFORME

41
Q

FRIED EGG APPEARANCE + CHICKEN-WIRE CAPILLARY PATTERN

A

OLIGODENDROGLIOMA (malignant adult)

42
Q

GFAP+

A

IMF of GLIAL CELLS (astrocytes) = CHILDREN PILOCYTIC ASTROCYTOMA + GLIOBLASTOMA MULTIFORME (ADULTS)

43
Q

CYSTIC LESION WITH MURAL THROMBUS on imaging

A

PILOCYTIC ASTROCYTOMA (Child, benign tumor)

44
Q

DROP METASTASIS to CAUDA EQUINA

A

MEDULLOBLASTOMA

45
Q

NEUROFIBROMATOSIS TYPE 2 BI-ALLELIC GENE INACTIVATION is associated with which CNS tumor?

A

SCHWANNOMA - TUMOR OF SCHWANN CELLS (most typically CN VIII), S100+

46
Q

Calcified tumor of white matter in FRONTAL LOBE

A

FRONTAL LOBE - think supratentorial adults

frOntal lObe = 2O’s = OLIGODENDROGLIOMA

47
Q

VEROCAY BODIES (ANTONI A [HYPERCELLULAR] + ANTONI B [HYPOCELLULAR])

A

Verocay bodies - alternating patterns think MUSIC = Need to hear = CN VIII
SCHWANNOMA (benign tumor of schwanna cells)

STEP 5: Neuro PATHOMA (6 decks)

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