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NMSP Exam 1 > Tumors > Flashcards

Tumors Flashcards

1
Q

Where do a majority of CNS tumors arise in children?

A
  • Posterior fossa –> Cerebellum or brainstem
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2
Q

Do CNS tumors travel outside the CNS?

A
  • Not usually
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3
Q

What can be seen in some pediatric tumors?

A
  • Spread through the CSF in the subarachnoid space
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4
Q

What is the most common CNS tumor overall?

A
  • Glioblastoma
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5
Q

What is the most common CNS tumor in children?

A
  • Embryonal –> Pilocytic astrocytoma and medulloblastoma
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6
Q

Where is the most common location for tumors in adults?

A
  • Supratentorial
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7
Q

What are the different grades of tumors?

A
  • Grade I: low proliferative potential, cure by resection
  • Grade II: Infiltrative, despite low proliferative potential –> some atypia
  • Grade III: Radiation or chemo –> Atypia and mitoses present
  • Grade IV: rapid pre and post op evolution, FATAL outcome –> microvascular proliferation and necrosis
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8
Q

What automatically makes a tumor a grade IV?

A
  • Necrosis
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9
Q

What are the four histologic parameters of grading gliomas?

A
  • Nuclear atypia
  • Mitoses
  • Microvascular proliferation
  • Necrosis
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10
Q

How is grading made for gliomas?

A
  • II = one parameter
  • III = two parameters
  • IV = 3 or 4 parameters
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11
Q

What are some grade I astrocytomas?

A
  • SEGA

- Pilocytic astrocytoma

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12
Q

What are some grade II astrocytomas?

A
  • Diffuse astrocytoma
  • PXA
  • Pilomyxoid astrocytoma
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13
Q

What is a grade III astrocytoma?

A
  • Anaplastic astrocytoma
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14
Q

What are some grade IV astrocytomas?

A
  • Glioblastoma
  • Giant cell GBM
  • Gliosarcoma
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15
Q

What grade is an oligodendroglioma?

A
  • Grade II or anaplastic grade III

- No grade IV so there is longer survival

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16
Q

What happens with risk for astrocytomas as people age?

A
  • Risk of higher grade as people age
  • Grade I usually seen in first decade
  • Grade II seen in 3rd-4th decade
  • Grade III seen in 5th decade
  • Grade IV seen in 6th decade and beyond
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17
Q

Where do astrocytomas usually affect?

A
  • White matter
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18
Q

What are some symptoms of a infiltrating astrocytoma (grade II to IV)?

A
  • Seizures
  • Headaches
  • Focal neuro deficits
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19
Q

What mutation makes for a better prognosis in infiltrating astrocytomas?

A
  • IDH-mutant is better than IDH wild type

- IDH R132H mutant protein IHC

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20
Q

What does an infiltrating astrocytoma look like grossly?

A
  • Poorly defined
  • Gray
  • Infiltrative tumors
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21
Q

What does an infiltrating astrocytoma look like microscopically?

A
  • Hypercellular

- Elongated, irregular hyperchromatic nuclei

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22
Q

Who is most likely affected by a pilocytic astrocytoma?

A
  • Children in the first decades of life
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23
Q

Where are pilocytic astrocytomas typically found?

A
  • Cerebellum
  • Third ventricle
  • Optic nerves
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24
Q

What mutation is seen in pilocytic astrocytomas?

A
  • IAA1549-BRAF gene
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25
Q

What predisposes children for pilocytic astrocytomas?

A
  • NF1 predisposes –> especially for optic nerves tumors
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26
Q

What do pilocytic astrocytomas look like?

A
  • Well circumscribed, often cystic with a mural nodule
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27
Q

What is the treatment for pilocytic astrocytomas?

A
  • Resection only
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28
Q

What does radiology show in pilocytic astrocytomas?

A
  • Discrete, contrast enhancing mural nodule
  • Lack of surrounding edema
  • Cyst
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29
Q

What is seen histologically for pilocytic astrocytomas?

A
  • Biphasic pattern –> loose glial with cystic changes and dense piloid tissue
  • Hair-like cells with long bipolar processes
  • Rosenthal fibers
  • EGBs
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30
Q

What genetic aberrations are seen in glioblastomas?

A
  • CDKN2A deletions
  • EGFR or PDGFR gene amplification
  • TP53 mutation –> resistance to apoptosis
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31
Q

Who is affected with primary glioblastoma? What mutations are seen?

A
  • Usually in older patients with no precursor lesion
  • IDH-wild type, Grade IV
  • TERT and EGFR
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32
Q

Who is affected with secondary glioblastoma? What mutations are seen?

A
  • Usually in younger patients with a low grade lesion (TP53)

- IDH1 (R132H better prognosis than wild type) and IDH2

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33
Q

What is seen in glioblastoma?

A
  • Contrast enhancing ring –> hypodense central necrosis imaging
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34
Q

What are the histologic hallmarks of glioblastomas?

A
  1. Necrosis –> serpentine pattern
  2. Pseudo-palisading of cells around necrosis
  3. Vascular/endothelial proliferation –> VEGF produced by malignant astrocytes in response to hypoxia
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35
Q

What age is affected by oligodendrogliomas?

A
  • People in 4th or 5th decades
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36
Q

What area do oligodendrogliomas affect?

A
  • Cerebral hemispheres

- Predilection for white matter

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37
Q

What are the symptoms of oligodendrogliomas?

A
  • Neurologic complaints for several years

- Calcifications usually restricted to the cortex, curvilinear or gyriform distribution

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38
Q

What do oligodendrogliomas look like histologically?

A
  • Perineuronal satellitosis
  • Perivascular aggregation and subpial accumulation of tumor cells
  • Perinuclear halos –> look like fried eggs
  • Delicate anastomosing capillaries –> looks like chicken wire
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39
Q

What are the most common mutation in oligodendrogliomas?

A
  • IDH1 and IDH2 –> Favorable prognosis

- 1P19Q loss –> favorable prognosis

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40
Q

What is seen in anaplastic oligodendroglioma? Prognosis?

A
  • Vascular hypertrophy and necrosis
  • Often retains geometric vascularity
  • Poor prognosis
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41
Q

Who is most affected by ependymomas?

A
  • Children in first two decades of life
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42
Q

Where are ependymomas usually found?

A
  • Fourth ventricle floor is most common site

- Spinal cord if found in adults

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43
Q

What do grade III ependymomas look like?

A
  • Usually supratentorial with mitoses, microvascular proliferation, necrosis
  • Round or oval nuclei
  • Fibrillary cytoplasmic processes extends to form meshwork
  • Ependymal rosettes –> more diagnostic than perivascular rosettes
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44
Q

What is seen in anaplastic ependymoma?

A
  • Increased cells
  • Increased mitosis
  • Palisading necrosis
  • +/- microvascular proliferation
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45
Q

What is the best predictor of anaplastic ependymoma?

A
  • Extent of surgical resection and molecular subtype
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46
Q

What is a myxopapillary ependymoma?

A
  • Filum terminale

- Cuboidal tumor cells arranged in a papillary pattern around mucin-fibrovascular cores

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47
Q

Where are subependymomas found?

A
  • Lateral or 4th ventricle under ependymal layer
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48
Q

What are some symptoms of subependymomas?

A
  • Slow growing, solid, +/- calcified
  • Usually asymptomatic and an incidental finding
  • Could cause obstructive hydrocephalus
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49
Q

Where are choroid plexus papillomas found?

A
  • Common in children

- Lateral ventricles

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50
Q

What can choroid plexus papillomas cause?

A
  • Hydrocephalus –> obstruction
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51
Q

What is a colloid cyst of the 3rd ventricle?

A
  • Found in young adults
  • Attached to roof of third ventricle
  • Can be rapidly fatal
  • Can be positional
52
Q

What can a colloid cyst of 3rd ventricle cause?

A
  • Obstruct foramen of monro causing noncommunicating hydrocephalus
53
Q

What is a ganglioglioma?

A
  • Mix of mature, neuronal, and glial cells
54
Q

How do gangliogliomas present?

A
  • Superficial lesions present as seizures; medical refractory epilepsy
  • Temporal lobes
55
Q

What is the activating mutation in gangliogliomas?

A
  • BRAF gene (V600E)
56
Q

What is dysembryoplastic neuroepithelial tumor (DNET) associated with?

A
  • Associated with epilepsy
57
Q

What is the treatment for gangliogliomas and DNET?

A
  • Surgical resection
58
Q

Where are DNETs found? What do they look like?

A
  • Superficial temporal lobes

- Multiple discrete mucin-rich intracortical nodules of small oligodendrocyte like cells

59
Q

What is the most common embryonal tumor?

A
  • Medulloblastoma
60
Q

What are some embryonal tumors?

A
  • Medulloblastoma
  • Atypical teratoid/rhabdoid tumor (AT/RT)
  • Primitive neuroectodermal tumor (PNET)
61
Q

What are the four groups of medulloblastomas?

A
  • WNT
  • SHH
  • Group 3
  • Group 4
62
Q

What medulloblastoma group has the best prognosis?

A
  • WNT
63
Q

What medulloblastoma group has the worst prognosis?

A
  • Group 3/4
64
Q

What mutation is seen in the WNT medulloblastoma?

A
  • Chr. 6 B-catenin
65
Q

What mutation is seen in the SHH medulloblastoma?

A
  • MCYN AMP
66
Q

What mutation is seen in group 3 medulloblastoma?

A
  • MCY and 17(17Q)
67
Q

What mutation is seen in group 4 medulloblastoma?

A
  • I17Q, no MYC or MYCN
68
Q

Where are WNT medulloblastomas found?

A
  • Anterior 4th ventricle or middle cerebral peduncle
69
Q

Where are SHH medulloblastomas found?

A
  • Vermis or lateral cerebellum
70
Q

What can be seen in medulloblastomas?

A
  • CSF flow can be occluded

- Propensity to spread into the subarachnoid space –> drop metz

71
Q

What do medulloblastomas look like grossly?

A
  • Well circumscribed
  • Gray friable
  • May involve leptomeninges
72
Q

What do medulloblastomas look like histologically?

A
  • Densely cellular, sheets of anaplastic cells

- Abundant mitoses –> KI-67 proliferation marker high)

73
Q

What is a part of the classic subtype of medulloblastomass?

A
  • Homer-Wright rosettes

- + Synaptophysin

74
Q

What is a part of the desmoplastic/nodular variant of medulloblastomas?

A
  • Internodular areas of stroma, collagen, and reticulin

- Pale islands of neuropil with neuronal differentiation

75
Q

What is a part of the large cell/anaplastic variant of medulloblastoma?

A
  • Large irregular vesicular nuclei
  • Prominent nucleoli
  • Cell wrapping
  • High mitotic/apoptotic rate
76
Q

What is an important feature of medulloblastoma?

A
  • Exquisitely radiosensitive –> WNT has 100% survival rate
77
Q

What tumors have the PTCH1 gene mutation?

A
  • Desmoplastic medulloblastoma

- Nevoid basal cell carcinoma syndrome

78
Q

Where are atypical teratoid/rhabdoid tumors found?

A
  • Posterior fossa or supratentorial
79
Q

What are atypical teratoid/rhabdoid tumors positive for?

A
    • EMA and vimentin
  • +/- Smooth muscle actin and keratins
  • Neg desmin and myoglobin
80
Q

What mutation is seen in atypical teratoid/rhabdoid tumors?

A
  • Chr 22; HSNF5/INI1
81
Q

Who is most likely affected with primary CNS lymphoma?

A
  • Immunosuppressed individuals –> AIDS or transplantation
82
Q

What are some qualities of primary CNS lymphoma?

A
  • Primary, often multifocal
  • Periventricular spread is common
  • B-cell origin CD20+
83
Q

What are people with primary CNS lymphoma typically infected with?

A
  • Latent infection of EBV
84
Q

What does the morphology of primary CNS lymphoma look like?

A
  • Accumulate around vessels

- “Hooping” = cells separated by reticulin + silver stain

85
Q

Where are germ cell tumors typically found?

A
  • Along midline

- Pineal and suprasellar

86
Q

Where are the metz typically found from germ cell tumors?

A
  • Gonadal germ cell tumors typically go to CNS
87
Q

What is a germinoma?

A
  • Resembles seminoma
  • Good response to XRT and chemo
  • AFP and BetaHCG can be used to track response
88
Q

When are meningiomas seen?

A
  • Females in their 3rd decade
  • Has a high predominance in women who are pregnant
  • Once baby is delivered, then meningioma reduces in size
89
Q

What happens in meningiomas?

A
  • They enlarge slowly and are reluctant to infiltrate the brain
  • Compresses the brain
90
Q

What is special about meningiomas?

A
  • Will penetrate the brain
91
Q

What causes meningiomas?

A
  • Radiation induced
92
Q

What mutation is seen in meningiomas?

A
  • NF2 –> multiple, loss Ch22Q
93
Q

What should be considered if there are multiple meningiomas are found?

A
  • NF2 diagnosis
94
Q

What mutations are seen in lower risk meningiomas?

A
  • TRAF7
  • KLF4
  • AKT1
  • SMO
95
Q

What mutations are seen in higher grade meningiomas?

A
  • TERT

- CDKN2A

96
Q

What do meningiomas look like?

A
  • Basselated dural mass that is rubbery and round
  • Compresses underlying brain
  • “En Plaque” sheetlike across the surface of the dura
97
Q

What do meningiomas look like histologically?

A
  • Calcified psammoma bodies –> sand like consistency

- Whorled clusters of monotonous cells

98
Q

Where are some places that meningiomas found?

A
  • Left lateral ventricle is favored

- Parasagittal, cerebral convexities, wing of sphenoid bone, sella, olfactory groove, foramen magnum, optic nerve

99
Q

What is seen in the atypical type of meningiomas?

A
  • Grade II
  • More aggressive
  • Increased recurrence
100
Q

What is seen in the anaplastic type of meningiomas?

A
  • Grade III
  • Resembles high grade carcinoma or sarcoma
  • De novo or malignant progression from lower grade
101
Q

What is the difference in mitoses between atypical meningioma and anaplastic meningioma?

A
  • Atypical –> ≥4 mitoses

- Anaplastic –> >20 mitoses

102
Q

What is a rare tumor but has frequent metz to the brain?

A
  • Choriocarcinoma
103
Q

What is a common malignancy but rarely metz to the brain?

A
  • Prostate
104
Q

What is a common site of metz in the brain?

A
  • Meninges

- Intraparenchymal = gray/white junction

105
Q

What is meningeal carcinomatosis?

A
  • Tumor nodules spread through subarachnoid –> cranial and spinal neuropathies from nerve root compression
  • Lung and breast most commonly
106
Q

What is subacute cerebellar degeneration?

A
  • PCA-1 antibody recognizes purkinje cells

- Destruction of purkinje cells, gliosis, and mild chronic inflammatory infiltrate

107
Q

Who typically has subacute cerebellar degeneration?

A
  • Women with ovarian, uterine, or breast carcinoma
108
Q

What is limbic encephalitis?

A
  • Subacute dementia

- Affects anterior and medial temporal lobe

109
Q

What other tumors are seen in cowden syndrome? What is the mutation?

A
  • Dysplastic gangliocytoma

- Mutation in PTN –> IK3/AKT signaling pathway

110
Q

What other tumors are seen in Li-fraumeni syndrome? What is the mutation?

A
  • Medulloblastomas

- Mutations in TP53

111
Q

What other tumors are seen in Turcot syndrome? What is the mutation?

A
  • Medulloblastoma or glioblastoma

- Mutations in APC or mismatch repair genes

112
Q

What other tumors are seen in Gorlin syndrome? What is the mutation?

A
  • Medulloblastoma

- Mutation in PTCH gene resulting in up regulation of SHH pathway

113
Q

What does the TSC1 gene encode for?

A
  • Hamartin
114
Q

What does the TSC2 gene encode for?

A
  • Tuberin
115
Q

What is seen in tuberous sclerosis?

A
  • Hamartomas –> cortical tubers
  • Retinal glial hamartomas
  • Renal angiomyolipomas
  • Cardiac rhabdomyomas
116
Q

What cutaneous lesions are seen in tuberous sclerosis?

A
  • Angiofibromas, subungual fibromas
  • Shagreen patches
  • Ash-leaf patches
117
Q

What is the mutation in von hippel lindau disease?

A
  • VHL on Ch3
118
Q

What does the VHL gene do?

A
  • Downregulates HIF-1 which regulates expression of VEGF

- Involved in the expression of erythropoietin causing polycythemia

119
Q

What is seen in von hippel lindau disease?

A
  • Hemangioblastomas of the CNS
  • Cysts of pancreas, liver, and kidneys
  • Renal cell carcinoma
  • Pheochromocytoma
120
Q

What is seen in neurofibromatosis type 1?

A
  • NF1 gene –> neurofibromin
  • Neurofibromas of peripheral nerves
  • Optic nerve gliomas
  • Lisch nodules
  • Cafe au lait spots
121
Q

What is seen in neurofibromatosis type 2?

A
  • NF2 gene –> merlin
  • Bilateral schwannomas (CN VIII)
  • Multiple meningiomas, ependymomas of the cervical spinal cord
122
Q

What is schwannomatosis?

A
  • Same frequency as NF2

- Multiple non vestibular schwannomas throughout the body or single region

123
Q

What is a schwannoma? What gene is affected?

A
  • S100+

- NF2 –> loss of merlin; even sporadic associated with NF2 gene

124
Q

What is seen in Antoni A schwannoma?

A
  • Dense
  • Eosinophilic spindle cells arranged intersecting fascicles
  • Verocay bodies –> palisading nuclei around “nuclear free zones”
125
Q

SWhat is seen in Antoni B schwannoma?

A
  • Hypocellular
  • Myxoid extracellular matrix
  • Microcyst formation

NMSP Exam 1 (12 decks)

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