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NMSP Exam 1 > Tumors > Flashcards

Tumors Flashcards

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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
What predisposes children for pilocytic astrocytomas?
- NF1 predisposes --> especially for optic nerves tumors
26
What do pilocytic astrocytomas look like?
- Well circumscribed, often cystic with a mural nodule
27
What is the treatment for pilocytic astrocytomas?
- Resection only
28
What does radiology show in pilocytic astrocytomas?
- Discrete, contrast enhancing mural nodule - Lack of surrounding edema - Cyst
29
What is seen histologically for pilocytic astrocytomas?
- Biphasic pattern --> loose glial with cystic changes and dense piloid tissue - Hair-like cells with long bipolar processes - Rosenthal fibers - EGBs
30
What genetic aberrations are seen in glioblastomas?
- CDKN2A deletions - EGFR or PDGFR gene amplification - TP53 mutation --> resistance to apoptosis
31
Who is affected with primary glioblastoma? What mutations are seen?
- Usually in older patients with no precursor lesion - IDH-wild type, Grade IV - TERT and EGFR
32
Who is affected with secondary glioblastoma? What mutations are seen?
- Usually in younger patients with a low grade lesion (TP53) | - IDH1 (R132H better prognosis than wild type) and IDH2
33
What is seen in glioblastoma?
- Contrast enhancing ring --> hypodense central necrosis imaging
34
What are the histologic hallmarks of glioblastomas?
1. Necrosis --> serpentine pattern 2. Pseudo-palisading of cells around necrosis 3. Vascular/endothelial proliferation --> VEGF produced by malignant astrocytes in response to hypoxia
35
What age is affected by oligodendrogliomas?
- People in 4th or 5th decades
36
What area do oligodendrogliomas affect?
- Cerebral hemispheres | - Predilection for white matter
37
What are the symptoms of oligodendrogliomas?
- Neurologic complaints for several years | - Calcifications usually restricted to the cortex, curvilinear or gyriform distribution
38
What do oligodendrogliomas look like histologically?
- Perineuronal satellitosis - Perivascular aggregation and subpial accumulation of tumor cells - Perinuclear halos --> look like fried eggs - Delicate anastomosing capillaries --> looks like chicken wire
39
What are the most common mutation in oligodendrogliomas?
- IDH1 and IDH2 --> Favorable prognosis | - 1P19Q loss --> favorable prognosis
40
What is seen in anaplastic oligodendroglioma? Prognosis?
- Vascular hypertrophy and necrosis - Often retains geometric vascularity - Poor prognosis
41
Who is most affected by ependymomas?
- Children in first two decades of life
42
Where are ependymomas usually found?
- Fourth ventricle floor is most common site | - Spinal cord if found in adults
43
What do grade III ependymomas look like?
- 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
44
What is seen in anaplastic ependymoma?
- Increased cells - Increased mitosis - Palisading necrosis - +/- microvascular proliferation
45
What is the best predictor of anaplastic ependymoma?
- Extent of surgical resection and molecular subtype
46
What is a myxopapillary ependymoma?
- Filum terminale | - Cuboidal tumor cells arranged in a papillary pattern around mucin-fibrovascular cores
47
Where are subependymomas found?
- Lateral or 4th ventricle under ependymal layer
48
What are some symptoms of subependymomas?
- Slow growing, solid, +/- calcified - Usually asymptomatic and an incidental finding - Could cause obstructive hydrocephalus
49
Where are choroid plexus papillomas found?
- Common in children | - Lateral ventricles
50
What can choroid plexus papillomas cause?
- Hydrocephalus --> obstruction
51
What is a colloid cyst of the 3rd ventricle?
- Found in young adults - Attached to roof of third ventricle - Can be rapidly fatal - Can be positional
52
What can a colloid cyst of 3rd ventricle cause?
- Obstruct foramen of monro causing noncommunicating hydrocephalus
53
What is a ganglioglioma?
- Mix of mature, neuronal, and glial cells
54
How do gangliogliomas present?
- Superficial lesions present as seizures; medical refractory epilepsy - Temporal lobes
55
What is the activating mutation in gangliogliomas?
- BRAF gene (V600E)
56
What is dysembryoplastic neuroepithelial tumor (DNET) associated with?
- Associated with epilepsy
57
What is the treatment for gangliogliomas and DNET?
- Surgical resection
58
Where are DNETs found? What do they look like?
- Superficial temporal lobes | - Multiple discrete mucin-rich intracortical nodules of small oligodendrocyte like cells
59
What is the most common embryonal tumor?
- Medulloblastoma
60
What are some embryonal tumors?
- Medulloblastoma - Atypical teratoid/rhabdoid tumor (AT/RT) - Primitive neuroectodermal tumor (PNET)
61
What are the four groups of medulloblastomas?
- WNT - SHH - Group 3 - Group 4
62
What medulloblastoma group has the best prognosis?
- WNT
63
What medulloblastoma group has the worst prognosis?
- Group 3/4
64
What mutation is seen in the WNT medulloblastoma?
- Chr. 6 B-catenin
65
What mutation is seen in the SHH medulloblastoma?
- MCYN AMP
66
What mutation is seen in group 3 medulloblastoma?
- MCY and 17(17Q)
67
What mutation is seen in group 4 medulloblastoma?
- I17Q, no MYC or MYCN
68
Where are WNT medulloblastomas found?
- Anterior 4th ventricle or middle cerebral peduncle
69
Where are SHH medulloblastomas found?
- Vermis or lateral cerebellum
70
What can be seen in medulloblastomas?
- CSF flow can be occluded | - Propensity to spread into the subarachnoid space --> drop metz
71
What do medulloblastomas look like grossly?
- Well circumscribed - Gray friable - May involve leptomeninges
72
What do medulloblastomas look like histologically?
- Densely cellular, sheets of anaplastic cells | - Abundant mitoses --> KI-67 proliferation marker high)
73
What is a part of the classic subtype of medulloblastomass?
- Homer-Wright rosettes | - + Synaptophysin
74
What is a part of the desmoplastic/nodular variant of medulloblastomas?
- Internodular areas of stroma, collagen, and reticulin | - Pale islands of neuropil with neuronal differentiation
75
What is a part of the large cell/anaplastic variant of medulloblastoma?
- Large irregular vesicular nuclei - Prominent nucleoli - Cell wrapping - High mitotic/apoptotic rate
76
What is an important feature of medulloblastoma?
- Exquisitely radiosensitive --> WNT has 100% survival rate
77
What tumors have the PTCH1 gene mutation?
- Desmoplastic medulloblastoma | - Nevoid basal cell carcinoma syndrome
78
Where are atypical teratoid/rhabdoid tumors found?
- Posterior fossa or supratentorial
79
What are atypical teratoid/rhabdoid tumors positive for?
- + EMA and vimentin - +/- Smooth muscle actin and keratins - Neg desmin and myoglobin
80
What mutation is seen in atypical teratoid/rhabdoid tumors?
- Chr 22; HSNF5/INI1
81
Who is most likely affected with primary CNS lymphoma?
- Immunosuppressed individuals --> AIDS or transplantation
82
What are some qualities of primary CNS lymphoma?
- Primary, often multifocal - Periventricular spread is common - B-cell origin CD20+
83
What are people with primary CNS lymphoma typically infected with?
- Latent infection of EBV
84
What does the morphology of primary CNS lymphoma look like?
- Accumulate around vessels | - "Hooping" = cells separated by reticulin + silver stain
85
Where are germ cell tumors typically found?
- Along midline | - Pineal and suprasellar
86
Where are the metz typically found from germ cell tumors?
- Gonadal germ cell tumors typically go to CNS
87
What is a germinoma?
- Resembles seminoma - Good response to XRT and chemo - AFP and BetaHCG can be used to track response
88
When are meningiomas seen?
- Females in their 3rd decade - Has a high predominance in women who are pregnant - Once baby is delivered, then meningioma reduces in size
89
What happens in meningiomas?
- They enlarge slowly and are reluctant to infiltrate the brain - Compresses the brain
90
What is special about meningiomas?
- Will penetrate the brain
91
What causes meningiomas?
- Radiation induced
92
What mutation is seen in meningiomas?
- NF2 --> multiple, loss Ch22Q
93
What should be considered if there are multiple meningiomas are found?
- NF2 diagnosis
94
What mutations are seen in lower risk meningiomas?
- TRAF7 - KLF4 - AKT1 - SMO
95
What mutations are seen in higher grade meningiomas?
- TERT | - CDKN2A
96
What do meningiomas look like?
- Basselated dural mass that is rubbery and round - Compresses underlying brain - "En Plaque" sheetlike across the surface of the dura
97
What do meningiomas look like histologically?
- Calcified psammoma bodies --> sand like consistency | - Whorled clusters of monotonous cells
98
Where are some places that meningiomas found?
- Left lateral ventricle is favored | - Parasagittal, cerebral convexities, wing of sphenoid bone, sella, olfactory groove, foramen magnum, optic nerve
99
What is seen in the atypical type of meningiomas?
- Grade II - More aggressive - Increased recurrence
100
What is seen in the anaplastic type of meningiomas?
- Grade III - Resembles high grade carcinoma or sarcoma - De novo or malignant progression from lower grade
101
What is the difference in mitoses between atypical meningioma and anaplastic meningioma?
- Atypical --> ≥4 mitoses | - Anaplastic --> >20 mitoses
102
What is a rare tumor but has frequent metz to the brain?
- Choriocarcinoma
103
What is a common malignancy but rarely metz to the brain?
- Prostate
104
What is a common site of metz in the brain?
- Meninges | - Intraparenchymal = gray/white junction
105
What is meningeal carcinomatosis?
- Tumor nodules spread through subarachnoid --> cranial and spinal neuropathies from nerve root compression - Lung and breast most commonly
106
What is subacute cerebellar degeneration?
- PCA-1 antibody recognizes purkinje cells | - Destruction of purkinje cells, gliosis, and mild chronic inflammatory infiltrate
107
Who typically has subacute cerebellar degeneration?
- Women with ovarian, uterine, or breast carcinoma
108
What is limbic encephalitis?
- Subacute dementia | - Affects anterior and medial temporal lobe
109
What other tumors are seen in cowden syndrome? What is the mutation?
- Dysplastic gangliocytoma | - Mutation in PTN --> IK3/AKT signaling pathway
110
What other tumors are seen in Li-fraumeni syndrome? What is the mutation?
- Medulloblastomas | - Mutations in TP53
111
What other tumors are seen in Turcot syndrome? What is the mutation?
- Medulloblastoma or glioblastoma | - Mutations in APC or mismatch repair genes
112
What other tumors are seen in Gorlin syndrome? What is the mutation?
- Medulloblastoma | - Mutation in PTCH gene resulting in up regulation of SHH pathway
113
What does the TSC1 gene encode for?
- Hamartin
114
What does the TSC2 gene encode for?
- Tuberin
115
What is seen in tuberous sclerosis?
- Hamartomas --> cortical tubers - Retinal glial hamartomas - Renal angiomyolipomas - Cardiac rhabdomyomas
116
What cutaneous lesions are seen in tuberous sclerosis?
- Angiofibromas, subungual fibromas - Shagreen patches - Ash-leaf patches
117
What is the mutation in von hippel lindau disease?
- VHL on Ch3
118
What does the VHL gene do?
- Downregulates HIF-1 which regulates expression of VEGF | - Involved in the expression of erythropoietin causing polycythemia
119
What is seen in von hippel lindau disease?
- Hemangioblastomas of the CNS - Cysts of pancreas, liver, and kidneys - Renal cell carcinoma - Pheochromocytoma
120
What is seen in neurofibromatosis type 1?
- NF1 gene --> neurofibromin - Neurofibromas of peripheral nerves - Optic nerve gliomas - Lisch nodules - Cafe au lait spots
121
What is seen in neurofibromatosis type 2?
- NF2 gene --> merlin - Bilateral schwannomas (CN VIII) - Multiple meningiomas, ependymomas of the cervical spinal cord
122
What is schwannomatosis?
- Same frequency as NF2 | - Multiple non vestibular schwannomas throughout the body or single region
123
What is a schwannoma? What gene is affected?
- S100+ | - NF2 --> loss of merlin; even sporadic associated with NF2 gene
124
What is seen in Antoni A schwannoma?
- Dense - Eosinophilic spindle cells arranged intersecting fascicles - Verocay bodies --> palisading nuclei around "nuclear free zones"
125
SWhat is seen in Antoni B schwannoma?
- Hypocellular - Myxoid extracellular matrix - Microcyst formation

NMSP Exam 1 (12 decks)

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