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NMSP 1 > Tumors > Flashcards

Tumors Flashcards

1
Q

In what general location of the brain are most adult brain tumors found vs. brain tumors in kids?

A

Adults - supratentorial

Kids - cerebellum and brainstem

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2
Q

What are common brain tumors in adults vs. kids?

A

Adults - glioblastoma and meningioma

Kids - pilocytic astrocytoma and medulloblastoma

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3
Q

True or False: brain tumors are the most common solid tumor in children

A

True; they are the second most common type of malignancy in children overall, after leukemia.

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4
Q

What should be at the top of your list of differential diagnoses in an adult w/ new onset seizure?

A

brain tumor

common adult brain tumor: glioblastoma

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5
Q

What are two characteristics features of a Grade IV tumor?

A
  • vascular proliferation

- necrosis

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6
Q

How are tumors graded using the four histological parameters of gliomas: atypia, mitoses, vascular proliferation, and necrosis?

A

one parameter: II (ex: atypia)

two parameters: III (ex: atypia and mitoses)

three/four parameters: IV

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7
Q

Pilocytic astrocytomas are commonly found in which age group and what grade are they?

A
  • children

- Grade I

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8
Q

Glioblastomas are commonly found in which age group and what grade are they?

A
  • adults

- Grade IV

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9
Q

How are age of patient and tumor grade related in astrocytomas?

A
  • the higher the patient age of typical onset, the higher the grade level of the tumor type
    ex: Grade II 40’s and 50’s; Grade III 60’s
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10
Q

Are astrocytomas usually white matter lesions or grey matter lesions?

A

White matter lesions

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11
Q

What age group is most common for pilocytic astrocytomas?

A

-younger than 20 yrs old

first two decades, same as ependymomas

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12
Q

What part of the brain is most common for pilocytic astrocytomas to be located?

A
  • cerebellum

- posterior fossa

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13
Q

What gene mutation predisposes patients to pilocytic astrocytomas?

A

NF1

-functional loss of neurofibromin in the tumor

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14
Q

What is the gross appearance of a pilocytic astrocytoma?

A
  • -well-circumscribed

- -cystic w/ a mural nodule

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15
Q

What do the cells of a pilocytic astrocytoma look like?

A

–hair-like cells w/ long bipolar processes

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16
Q

Do you typically see Rosenthal Fibers in a Pilocytic Astrocytoma?

A

Yes!

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17
Q

Besides Rosenthal Fibers, what other structure do you see on histology of Pilocytic Astrocytomas?

A

Eosinophilic Granular Bodies (EGB’s)

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18
Q

Are pilocytic astrocytomas GFAP positive or negative?

A

GFAP positive

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19
Q

What is the most common primary brain neoplasm?

A

glioblastoma (Grade IV)

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20
Q

What is a primary glioblastoma vs a secondary glioblastoma?

A
  • primary glioblastomas appear as a new onset dz in older individuals
  • secondary glioblastomas occur in younger pts as a results of progression of a lower-grade astrocytoma
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21
Q

What genetic changes are seen in primary glioblastomas?

A
  • mutations of PTEN suppressor gene
  • deletions of chromosome 10
  • amplification of EGFR oncogene
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22
Q

What genetic changes are seen in secondary glioblastomas?

A

-mutations of p53

  • point mutations of IDH1 and IDH2 (isocitrate dehydrogenase)
  • -mutant form of IDH1 (R132H) has a better outcome than the wild type IDH1
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23
Q

What is seen on imaging and morphology of a glioblastoma?

A
  • -contrast ring-enhancing
  • -central necrosis
  • -crosses the corpus callosum
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24
Q

What is seen on histology of glioblastomas?

A
  • necrosis in a serpentine pattern in hypercellular areas
  • pseudo palisading
  • vascular/endothelial proliferation
  • -VEGF production d/t hypoxia
  • -glomeruloid bodies bulging into vascular lumen
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25
Q

Where in the brain do oligodendrogliomas (Grade II) usually occur?

A
  • primarily in the cerebral hemispheres

- predilection for white matter

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26
Q

What age group is most commonly affected by oligodendrogliomas?

A

-adults in their 50’s and 60’s

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27
Q

What is seen on morphology of oligodendrogliomas?

A

-calcification of the cortex in a gyriform distribution

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28
Q

What is seen on histology of oligodendrogliomas?

A
  • perineuronal satellitosis (tumor cells collect around neurons)
  • perivascular aggregation (chicken-wire pattern of vasculature, delicate anastamosing capillaries)

Note: remember oligodendrocytes look like “fried eggs” on H/E stains d/t the perinuclear halo artifacts

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29
Q

What is the most common gene mutation in oligodendrogliomas?

A
  • -IDH1 and IDH2 (present in 90% of cases)
  • –hold a favorable prognosis

–1p and 19q co-deletions (80% of cases)

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30
Q

What is notable about treatment of oligodendrogliomas with a 1p and 19q loss?

A

–consistent, long-lasting response to chemo and radiation

–tumors w/o the 1p and 19q loss appear to be resistant to chemo regimens

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31
Q

What are the characteristics of an anaplastic oligodendrogliomas (Grade III)?

A
  • increased nucleus:cytoplasm ratio
  • increased mitosis
  • increased cellularity
  • can be found in nodules within Grade II tumors
  • poor prognosis (but still better than glioblastomas)
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32
Q

What age group is typically affected by ependymomas?

A

-younger than 20yrs old

first two decades, same as pilocytic astrocytomas

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33
Q

What is the most common site in the brain for ependymomas?

A

-fourth ventricle (arising from the floor)

  • spinal cord in adults
  • -frequent w/ neurofibromatosis type 2 (NF2)
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34
Q

What is the gross morphology of ependymomas in the fourth ventricle?

A
  • discrete
  • exophytic
  • enhancing
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35
Q

What is seen on histology of ependymomas?

A
  • ependymal rosettes (true rosettes)
  • perivascular rosettes

–true ependymal rosettes are more diagnostic (tubular structure w/ central canal)

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36
Q

What are the clinical features of ependymomas?

A
  • hydrocephalus d/t 4th ventricle obstruction
  • dissemination into the CSF (poor prognosis)
  • 5yr survival in young children is less than 50%
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37
Q

What type of tumor is a medulloblastoma?

A
  • malignant embryonal tumor in kids
  • -neuroectoderm in origin; very poorly differentiated

-located exclusively in the cerebellum (midline)

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38
Q

What are the four groups of molecular alterations in medulloblastomas?

A
  • WNT signaling pathway mutation (chr 6 monosomy)
  • SHH signaling pathway mutation (MYC amplification)
  • i17q w/ MYC (infants and young kids; worst outcome)
  • i17q w/o MYC (poor prognosis)
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39
Q

Which molecular alteration in medulloblastoma has the best prognosis and which has the worst prognosis?

A
  • WNT is best (90% 5yr survival rate)

- i17q w/MYC amplification is the worst prognosis

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40
Q

What is a complication of rapid growth of a midline cerebellar medulloblastoma?

A

-hydrocephalus d/t occlusion of CSF flow

41
Q

What do you seen on histology of a medulloblastoma?

A
  • sheets of anaplastic cells
  • abundant mitoses
  • Homer Wright rosettes (neuropil in center)
42
Q

What is a common complication of tumor cell dissemination into the CSF that can happen w/ a medulloblastoma?

A

-nodular masses, sometimes as far as the cauda equina

aka “drop metastases

43
Q

What is notable about the treatment of medulloblastomas?

A

-they are exquisitely radiosensitive

44
Q

What tumor resembles a medulloblastoma that occurs in the cerebral hemispheres?

A
  • CNS PNET
  • -PRIMITIVE neuroectodermal tumor)

-poorly differentiated

45
Q

Where in the brain do AT/RT (atypical teratoid/rhabdoid tumors) typically occur?

A
  • posterior fossa

- supratentorial

46
Q

What types of cells comprise an AT/RT?

A
  • divergent differentiation
  • -epithelial
  • -mesenchymal
  • -neuronal
  • -glial
  • -rhabdoid
47
Q

What proteins are present in rhabdoid cell cytoplasm that test positive in AT/RT’s?

A

+epithelial membrane antigen (EMA)
+vimentin

+smooth muscle actin and keratins
NO desmin or myoglobin

48
Q

What do rhabdoid cells look like on H and E stain?

A
  • eosinophilic cytoplasm
  • sharp borders
  • eccentric nucleus
49
Q

What is the most common genetic mutation in AT/RT (atypical teratoid/rhabdoid tumor)?

A
  • mutation on chr 22 in 90% of cases

- hSNF5/INI1 gene

50
Q

What is the age most commonly affected by AT/RT’s and what is the prognosis?

A
  • nearly all tumors occur before the age of 5

- most pts live less than 1yr after Dx

51
Q

What is the most common CNS neoplasm in immunocompromised individuals, such as those with AIDS?

A

-Primary CNS lymphoma

52
Q

True or False: primary CNS lymphoma often spreads outside of the CNS into LN’s and bone marrow

A

False; and the converse is the same. Lymphomas arising outside the CNS rarely involve brain parenchyma.

53
Q

What are the main locations of Primary CNS Lymphoma and the major cell type involved?

A
  • multifocal
  • periventricular spread is common

-usually B cells (CD20+) w/ high growth fraction
in immunocompromised, EBV is normally positive

54
Q

Where do the tumor cells of a primary CNS lymphoma tend to accumulate?

A

-malignant cells infiltrate the brain parenchyma and accumulate around blood vessels

55
Q

What is seen on a silver stain of Primary CNS lymphoma?

A

“hooping”

-cells are separated by reticulin

56
Q

Where in the brain do primary germ cell tumors occur?

A

-along the midline

57
Q

What ages and sex is most commonly affected by germ cell tumors in the brain?

A
  • pineal location has a male predominance
  • also occur in a suprasellar location
  • 90% occur in those younger than 20yrs
58
Q

What should be ruled out if a germ cell tumor is found in the brain?

A

-need to r/o metastasis from a gonadal germ cell tumor, since metastasis to the brain is common

59
Q

What is a germ cell tumor’s typical response to treatment?

A
  • good response to radiation and chemo

- alpha fetoprotein and hcg are used to track the tumor’s response to treatment

60
Q

What is the most common type of pineal tumor?

A

germinoma

61
Q

What are two kinds of pineal parenchyma tumors?

A

Pineocytoma:

  • neuronal differentiation,
  • low grade, adults

Pineoblastoma:

  • necrosis, mitoses, RB gene
  • high grade, kids
  • spreads throughout the CNS
62
Q

When do meningiomas (Grade I) usually occur?

A

-occur after age 30

63
Q

True or False: meningiomas commonly infiltrate the brain

A

False; they enlarge slowly and don’t typically infiltrate the brain, but they will cause compression of it.

Meningiomas WILL penetrate BONE.

64
Q

What is the common etiology of meningiomas?

A

radiation-induced from cancer treatment decades prior

65
Q

What is the most common cytogenic abnormality in meningiomas?

A
  • loss of 22q in 55% of cases
  • -includes 22q12 (NF2) which codes for merlin

-tend to be higher grade tumors

66
Q

What should be considered as a diagnosis in someone presenting with multiple meningiomas?

A

Neurofibromatosis Type 2

67
Q

After NF2 gene, what is another cytogenic abnormality in meningiomas?

A

TRAF-7 (TNF receptor associated factor 7)

-tends to be lower grade tumors

68
Q

What is the typical morphologic presentation of a meningioma?

A
  • -rounded, well-defined mass with dural base
  • -intraventricular or parasagittal
  • -bosselated (bunches of balls like a blackberry)
  • -contain calcified psammoma bodies

–or “en plaque” that spread like a sheet along dura

69
Q

What is the cell of origin in meningiomas?

A
  • arises from the meningothelial cells of the arachnoid

- EMA+ (epithelial membrane antigen)

70
Q

What is seen on histology of meningiomas?

A
  • whorled clusters of monotonous cells
  • psammoma bodies (calcification of nests of cells)

-the secretory type has PAS+ intracytoplasmic droplets

71
Q

What are the characteristics of an Atypical Meningiomas (Grade II)?

A
  • higher rate of recurrence
  • may require radiation after surgery
  • 4+ mitoses per 10 HPF

-“clear cell” and “choroid” are also in this category because they’re more aggressive

72
Q

What are the characteristics of an Anaplastic (malignant) meningioma (Grade III)?

A

-20+ mitoses per 10 HPF

Papillary Subtype:
-pleomorphic cells arranged around fibrovascular core

Rhabdoid Subtype:
-tumor cell sheets w/ hyaline eosinophilic cytoplasm

73
Q

What type of hormone receptor is common for meningiomas to display and what is a consequence of this?

A
  • 70% express progesterone receptors

- may grow more rapidly during pregnancy

74
Q

What type of cancer accounts for more than half of metastases to the brain?

A
  • metastatic carcinomas

- -most commonly lung and breast carcinomas

75
Q

What is an example of a rare tumor that frequently metastasizes to the brain?

A

choriocarcinoma

76
Q

What is an example of a very common cancer that rarely metastasizes to the brain?

A

prostate cancer

77
Q

What part of the brain is a frequent site of metastasis?

A

meninges

Meningeal Carcinomatosis: tumor nodules studding the surface of the brain, spinal cord, and intradural nerve roots (most commonly associated w/ lung and breast CA)

78
Q

What is the morphology of intraparenchymal metastases?

A
  • form sharply demarcated masses
  • occur at the grey to white junction
  • often surrounded by edema
79
Q

What are the characteristics of Subacute Cerebellar Degeneration (a paraneoplastic syndrome)?

A
  • -gliosis, destruction of Purkinje cells
  • -PCA-1 antibody
  • -predominantly ovarian, uterine, or breast carcinoma
80
Q

What are the characteristics of Limbic Encephalitis (a paraneoplastic syndrome)?

A

–ANNA-1 Ab (Anti-Hu) recognizes neuronal nuclei in CNS and PNS, most common from small cell lung CA

–NMDA receptor Ab cross reacts w/ hippocampus

–VGKC-complex Ab recognizes voltage-gated K+ channels; associated w/ peripheral neuropathy

81
Q

True or False: symptoms of limbic encephalitis occur before any malignancy is suspected

A

True

Limbic encephalitis characterized by: subacute dementia, perivascular inflammatory cuffs, microglial nodules, neuronal loss, gliosis (most evident in anterior/medial temporal lobe)

82
Q

What eye movement disorder may be found as a paraneoplastic syndrome?

A

–opsoclonus (uncontrolled, irregular, non-rhythmic eye mvmt)

–neuroblastoma in kids

83
Q

What is Lambert-Eaton Myasthenic Syndrome (a paraneoplastic syndrome)?

A

–Ab’s against voltage-gated Ca+ channels

84
Q

What is the treatment for paraneoplastic syndromes?

A
  • immunotherapy to remove the circulating Ab’s

- tumor removal

85
Q

What are the associated features of Neurofibromatosis Type 1 (more common than Type 2)?

A
  • pheochromocytoma
  • cafe au lait spots
  • optic nerve glioma
  • pigmented nodules of the iris (Lisch nodules)
  • neurofibromas of peripheral nerve
86
Q

What are the associated neoplasms of Von Hippel-Lindau Disease (VHL)?

A
  • pheochromocytoma
  • renal cell carcinoma
  • hemangioblastoma
  • pancreatic endocrine neoplasm
87
Q

What is the inheritance of Von Hippel-Lindau Disease?

A

autosomal dominant

  • VHL chr3p25q3 (tumor suppressor gene) mutation
  • -downregulates HIF-1, which regulations VEGF
  • -regulates EPO (creates polycythemia)
88
Q

What is the inheritance of Neurofibromatosis Type 1?

A

-autosomal dominant

89
Q

What are the characteristics of Neurofibromatosis Type 2?

A
  • B/L schwannomas of CN VIII
  • multiple meningiomas
  • multiple ependymomas
90
Q

What are the characteristics of Cowden Syndrome?

A
  • dysplastic gangliocytoma of the cerebellum

- PTEN mutation causes PI3K/AKT signal pathway

91
Q

What is Li-Fraumeni Syndrome?

A
  • medulloblastomas

- p53 mutations

92
Q

What is Turcot Syndrome?

A
  • medulloblastoma
  • glioblastoma
  • APC mutations or mismatch repair genes
93
Q

What is Gorlin Syndrome?

A
  • -medulloblastoma

- -PTCH mutations (up-regulation of SHH pathway)

94
Q

What is the inheritance of Tuberous Sclerosis and the neuro symptoms?

A

-autosomal dominant

  • seizures (d/t cortical epileptogenic tubers)
  • autism, mental retardation
95
Q

What are the genes involved in Tuberous Sclerosis?

A
  • TSC1 (chr 9q34 for hamartin)

- TSC2 (chr 16q13.3 for tuberin)

96
Q

What are the physical symptoms of Tuberous Sclerosis?

A
  • benign hamartomas
  • subependymal nodules (“candle-guttering”)
  • SEGA (subependymal giant cell astrocytomas)
  • renal angiomyolipomas
  • cardiac rhabdomyomas
  • cutaneous lesions
  • -Shagreen Patches (localized thickening)
  • -Ash Leaf Patches (hypopigmented areas)
97
Q

What are the characteristics of a Schwannoma (a peripheral nerve sheath tumor)?

A
  • S100+
  • acoustic neuroma of CN VIII … tinnitus, hearing loss
  • NF2 (loss of merlin)
  • Antoni A areas: spindle cells, verocay bodies (palisading nuclei around nuclear free zones)
  • Antoni B areas: hypocellular w/ myxoid EC matrix
98
Q

Are the majority of Malignant Peripheral Nerve Sheath Tumors high grade or low grade?

A
  • 85% are high grade

- about 50% occur in NF1 pts

99
Q

What is the “divergent differentiation” that is exhibited in malignant peripheral nerve sheath tumors?

A
  • glandular, cartilaginous, osseous, rhabdomyoblastic morphology d/t differentiation in focal areas
  • Triton Tumors exhibit rhabdomyoblastic areas

NMSP 1 (9 decks)

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