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2. Neuro exam 2 > Martin: Tumors > Flashcards

Martin: Tumors Flashcards

1
Q

Which grade of tumor is considered infiltrative?

A

Grade II

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2
Q

In which decade of life are Grade II, III, and IV Astrocytomas found?

A
  • Grade II: usually 3rd-4th decase
  • Grade III: usually 5th decade
  • Grade IV: usually 6th decade and beyond
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3
Q

Pilocytic Astrocytomas generally occur during what decades of life?

Where in brain do they typically occur?

A
  • First 2 decades of life
  • Cerebellum and floor/walls of 3rd ventricle, occasionally cerebral hemispheres
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4
Q

What are the distinguishing morphological characteristics of Pilocytic Astrocytomas?

A

- Well-circumscribed, often CYSTIC w/ a mural nodule

- Biphasic pattern

  • Hair-like cells w/ long bipolar processes
  • Rosenthal fibers
  • Eosinophilic granular bodies (EGBs)
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5
Q

Which disease predisposes patients to Pilocytic Astrocytomas and due to what?

A

NF-1 due to functional loss of neurofibromin

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6
Q

Among the higher grade glioblastomas, presence of what mutation is associated with a better outcome?

A

Mutant form of IDH1

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7
Q

What is the histologic pattern characteristically produced by Glioblastomas?

A

Pseudo-palisading: tumor cells collecting along the edges of necrotic regions

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8
Q

Necrosis in glioblastoma often occurs in a ________ pattern in areas of hypercellularity

A

Serpentine

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9
Q

Which additional features differentiate a glioblastoma from anaplastic astrocytomas?

A

Necrosis and vascular (glomeruloid type)/endothelial cell proliferation

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10
Q

What characteristic of Glioblastomas is seen on MRI?

A

Ring-enhacing lesions with central necrosis

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11
Q

Which tumor often crosses the corpus callosum and produces a “butterfly” appearance

A

Glioblastoma

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12
Q

Contrast ring enhancing lesions, with hypodense central necrosis is characteristic of what kind of tumor?

A

Glioblastoma

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13
Q

Which brain tumor appears as a cystic mass with a mural nodule?

A

Pilocystic astrocytoma

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14
Q

When are Oligodendrogliomas most commonly seen (decades)?

How are they graded?

A
  • Most common in fourth and fifth decades
  • Grade II/IV
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15
Q

What are the most common mutations seen in Oligodendrogliomas; which have a better prognosis?

A
  • IDH1 and IDH2 (better prognosis)
  • 1p19q (favorable prognosis)
  • CDKN2A
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16
Q

What are the distinguishing morphological characteristics of Oligodendrogliomas?

A
  • CALCIFICATION
  • Perinuclear halos, “fried eggs” and delicate anastomosing capillaries, “chicken wire”
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17
Q

Which features distinguish an Anaplastic Oligodendroglioma (III/IV) from an Oligodendroglioma?

A

Vascular hypertrophy, necrosis, and nuclear anaplasia

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18
Q

During which decades of life are Ependymomas most commonly seen and where are they most commonly located?

A
  • First 2 decades
  • Fourth ventricle = most common site
  • Spinal cord = most common for adults
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19
Q

Which chromosome is the NF2 gene found on?

A

Chromosome 22

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20
Q

Well circumscribed tumors in the brain that often have cysts, focal hemorrhage, and calcification generally describes?

A

Oligodendrogliomas

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21
Q

A tumor displaying uniform populations of cells w/ round or oval nuclei w/ abundant clumped chromatin and the appearance of perivascular rosettes is often what type?

A

Ependymoma

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22
Q

Which rosette type found in ependymomas are more diagnostic?

A

Ependymal rosettes (true rosettes) = MORE diagnostic than perivascular rosettes

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23
Q

What is a frequent clinical manifestation of posterior fossa ependymomas?

A

Hydrocephalus (non-communicating) secondary to progressive obstruction of the 4th ventricle

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24
Q

Homer-Wright rosettes are most often seen with what 2 brain tumors?

A

1) Medulloblastoma
2) Neuroblastoma

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25
Q

Choroid plexus papillomas are most often found where in children and adults?

A
  • In children – lateral ventricles
  • In adults – fourth ventricle
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26
Q

Who is most often affected by Non-neoplastic Colloid Cysts of the 3rd Ventricle?

What is the clinical manifestation?

A
  • Young adults
  • Found attached to roof of 3rd ventricle, can obstruct foramina of Monro = non-communicating hydrocephalus
  • Can be rapidly fatal
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27
Q

What is the most common malignant embryonal CNS tumor in children?

A

Medulloblastoma (IV/IV)

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28
Q

Medulloblastomas are often located where?

A

Midline of the Cerebellum

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29
Q

Medulloblastomas that occur in older children tend to have what genetic mutations?

A
  • Mutations in WNT signaling pathway
  • Monosomy of chromosome 6
  • Nuclear expression of β-catenin
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30
Q

What is the prognosis of the WNT type of medulloblastomas?

A

Best prognosis: with 90% 5-year survival

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31
Q

Medulloblastomas with the second best prognosis, behind the WNT type, tend to occur in infants-young children and are associated with what mutations?

A
  • Mutations in SHH pathway
  • May also have MYCN amplification
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32
Q

Group 3 medulloblastomas (worst prognosis) tend to occur in infants-young children and are associated with what mutations?

A
  • MYC amplification
  • Isochromosome 17 (i17q)
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33
Q

What are the mutations associated with group 4 Medulloblastomas and what is the prognosis?

A
  • i17q cytogenic alterations, classic or large cell histology
  • WITHOUT MYC amplification
  • Sometimes with MYCN amplification
  • Intermediate prognosis, in general i17q signals a worse prognosis
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34
Q

What is a common complication due to Medulloblastomas propensity to form linear chains and infiltrate?

A
  • Dissemination through CSF is common complication, giving rise to nodular masses some distance from the priamry tumor (i.e., as far as the cauda equina) – Drop Metastases
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35
Q

How well do Medulloblastomas respond to treatment?

A
  • Exquisitely radiosensitive
  • Total excision and irradiation, 5 year survival of 75%
36
Q

What are CNS supratentorial primitive neuroectodermal tumors (CNS PNET)?

A

A distinct type of tumor that is poorly differentiated and resembling a medulloblastoma

37
Q

Atypical Teratoid/Rhabdoid Tumors are most commonly seen in whom and where are they seen in the brain?

Benign or malignant?

A
  • Highly malignant tumor (grade IV) of young children (most often before age 5)
  • Posterior fossa and supratentorial compartments
38
Q

Which chromosome is most often altered in Atypical teratoid/Rhaboid tumors and is considered a hallmark; what specific gene?

A
  • Chromosome 22
  • hSNF5/INI1
  • Deletion of the locus and loss of nuclear staining for INI1 protein seen in majority
39
Q

What is the most common CNS neoplasm in immunosuppressed patients, such as those with AIDS or following transplantation?

A

Primary CNS lymphoma

40
Q

Vast majority of primary brain lymphomas are of _____origin (immune cell)

A

B-cell CD20+

41
Q

In the setting of immunosuppression, the cells in nearly all primary brain lymphomas are latently infected by which virus?

In the setting of organ transplantation, may be associated with a systemic what?

A
  • Epstein-Barr virus
  • Systemic post-transcriptional lymphoproliferative disorder
42
Q

What is the morphology of the lesions associated with Primary CNS Lymphomas?

Which stain shows their characteristic pattern?

A
  • Frequently mutliple and often involve deep gray matter as well as white matter and cortex; accumulate around vessels
  • Reticulin + silver stain show infiltrating cells separated from one another in a pattern called “hooping.”
43
Q

Primary brain germ cell tumors occur along where?

Most commonly in what locations?

A
  • Along the MIDLINE!
  • Most commonly in the pineal (male predominance) and suprasellar regions
44
Q

During what decades do the majority of Primary Brain Germ Cell tumors occur and who is affected the most?

A
  • 90% during first 2 decades
  • Japanese have highest incidence
45
Q

Germ cell tumors in the pineal region show a strong predominance in which sex?

A

Males

46
Q

Which markers of CNS germ cell tumors may be helpful in assisting with diagnosis and tracking response to therapy?

A
  • α-fetoprotein
  • β- hCG
47
Q

Metastasis of which type of germ cell tumor to the CNS is common?

What is the significance of this in regards to classifying the tumor?

A

Gonadal germ cell tumors

*Why the presence of a non-CNS primary tumor must be excluded before a diagnosis of primary germ cell tumor of the CNS is made!

48
Q

The tumor that is histologically similar to a seminoma in the testis is reffered to what in the CNS?

A

Germinoma

49
Q

What is the response of primary brain germ cell tumors to radiation therapy/chemotherapy?

A

Good response

50
Q

Differentiate Pineocytomas from Pineoblastomas in regards to grade and and who they most commonly affect.

A
  • Pineocytomas = LOW grade and more common in adults
  • Pineoblastomas = HIGH grade and more common in kids
51
Q

Pineoblastomas occur with increased frequency in individuals with which germ line mutations?

A

Germ line mutations in RB

52
Q

What is the most common pineal tumor?

A

Germinoma

53
Q

What is a risk factor for the development of Meningiomas?

A

Prior radiation therapy to the head and neck, typically decades earlier

54
Q

What is the severity of Meningiomas, who are they commonly seen in and where are they found?

A
  • Benign tumors of adults
  • Usually attached to the dura; found along any of the external surfaces of the brain as well as ventricular system
55
Q

What is the most common cytogenic abnormality seen in Meningiomas?

A
  • Loss of chromosome 22, especially the long arm (22q)
  • Including the region that harbors the NF2 gene, which encodes the protein merlin
56
Q

Higher grade Meningiomas are associated with what mutations?

A

NF2 mutations, loss of chromosome 22, and evidence of chromosomal instability

57
Q

What is the most common mutation seen in Meningiomas without NF2 mutations?

What is the histologic grade of this type?

A
  • TNF-receptor associated 7 (TRAF7)
  • Lower histologic grade
58
Q

Which tumors are often seen growing en plaque, in which the tumor spreads in a sheet like fashion along the surface of the dura?

A

Meningiomas

59
Q

Which tumor of the CNS is characterized by whorled clusters of monotonous cells; psammoma bodies?

A

Meningioma

60
Q

What is the typical patient presentation for a Meningioma?

A

Present with vague non-localizing signs and symptoms or with focal findings due to compression of underlying brain

61
Q

Which receptors may be expressed by Meningiomas and which patient population can this potentially affect?

A
  • Progesterone and Estrogen receptors
  • Pregnancy increases symptomatic presentation NOT incidence
62
Q

What are the 5 most common primary sites for potential metastasis to the brain?

A
  1. Lung
  2. Breast
  3. Skin (melanoma)
  4. Kidney
  5. GI
63
Q

Which rare tumor has a high likelihood of metastasing to the brain?

A

Choriocarcinomas

64
Q

Meningeal carcinomatosis with tumor nodules studding the surface of the brain, spinal cord, and intradural nerve roots is most commonly associated with carcinoma of the _______ and _______.

A

Lung and Breast

65
Q

Subacute cerebellar degeneration (paraneoplastic syndrome) is associated with destruction of which structures?

Which antibody?

Which cancers?

A
  • Destruction of Purkinje cells, gliosis, and a mild chronic inflammatory cell infiltrate
  • PCA-1 antibody (anti-Yo)
  • Women w/ ovarian, uterine, or breast carcinoma
66
Q

Which antibody is most commonly associated with small cell carcinoma of the lung and may cause limbic encephalitis (paraneoplastic)?

A

ANNA-1 antibody (anti-Hu) recognized neuronal nuclei in the CNS and PNS

67
Q

Which antibody is most commonly associated with ovarian teratomas and may cause limbic encephalitis (paraneoplastic)?

A

NMDA receptor antibody cross-reacts with hippocampal neurons

68
Q

Which antibody is most commonly associated with peripheral neuropathy and may cause limbic encephalitis (paraneoplastic)?

A

VGKC-complex antibody: voltage-gated potassium channel

69
Q

What is an important consideration in regards to limbic encephalitis and malignancy?

A

The syndrome appears before any malignancy is suspected

70
Q

Opsoclonus (eye movement disorder) in children is most commonly associated with what malignancy?

A

Neuroblastoma

71
Q

Cowden syndrome is associated with what tumor; due to what mutation?

A
  • Dysplastic gangliogliocytoma of the cerebellum (Lhermitte-Duclos disease)
  • Mutations in PTEN resulting in PI3K/AKT signaling pathway activity
72
Q

Li-Fraumeni syndrome is associated with what tumor; due to what mutation?

A
  • Medulloblastomas
  • Mutations in TP53
73
Q

Turcot syndrome is associated with what tumor; due to what mutation?

A
  • Medulloblastoma or Glioblastoma
  • Mutations in APC or mismatch repair genes
74
Q

Gorlin syndrome is associated with what tumor; due to what mutation?

A
  • Medulloblastoma
  • Mutations in PTCH gene – upregulation of SHH signaling pathways
75
Q

What is the inheritance pattern for Tuberous Sclerosis Complex?

A

Autosomal Dominant

76
Q

Tuberous Sclerosis Complex is characterized by the development of what?

Most frequent clinical manifestations are?

A
  • Development of hamartomas and benign neoplasms involving the brain and other tissues
  • Seizures, autism, and mental retardation
77
Q

What are the 2 tuberous sclerosis locuses; associated chromosome; and protein each encodes

Which is more commonly mutated?

A
  • TSC1 on chromosome 9q34 encodes hamartin
  • TSC2 on chromosome 16p13.3 encodes tuberin = Most commonly mutated
78
Q

Which tumors are associated with the Tuberous Sclerosis Complex?

A
  • Renal angiomyolipomas
  • Cardiac rhabdomyomas
  • Giant-cell astrocytomas
79
Q

What are the cutaneous lesions associated with the Tuberous Sclerosis Complex?

A
  • Shagreen patches (localized cutaneous thickenings)
  • Ash-leaf patches (hypopigmented areas)
80
Q

Multiple drop like masses associated with clusters of large tumor cells beneath the ventricular surface that buldge into the ventricular system and are known as candle-guttering is associated with?

A

Tuberous sclerosis – giant cell astrocytomas

81
Q

Von Hippel-Lindau Disease is associated with the development of which malignancies?

A
  • Hemangioblastomas of the CNS (cerebellum and retina)
  • Renal cell carcinoma
  • Pheochromocytoma
82
Q

What is the inheritance pattern of Von Hippel-Lindau disease; which gene and chromosome are involved?

What is the function of the protein associated with this gene?

A
  • AD
  • VHL = tumor suppressor gene on chromosome 3p25.3
  • VHL is component of ubiquitin ligase complex that down-regulates HIF-1 –> involved in expression of VEGF and erythropoietin
83
Q

What is responsible for the polycythemia observed in hemangioblastomas associated with Von Hippel-Lindau Disease?

A

VHL is involved in regulating expression of erythropoietin —> Polycythemia

84
Q

What is the mnemonic using VHL for Von Hippel-Lindau Disease?

A
  • V = VHL gene
  • H = Hemangioblastoma
  • L = Lots of catecholamines = pheochromocytoma

*VHL = 3 letters = RCC (renal cell carcinoma)

*VHL = 3 letters = chromosome 3

85
Q

NF1 is more common and is characterized by which malignancies and other findings?

A
  • Neurofibromas of peripheral nerve
  • Gliomas of optic nerve
  • Pigmented nodules of the iris (L**isch nodules)
  • Cutaneous hyperpigmented macules (cafe au lait spots)
86
Q

NF2 is most commonly characterized by which malignancies?

A
  • Bilateral schwannomas of CN VIII
  • Multiple meningiomas
  • Gliomas may also occur and are typically ependymomas of the spinal cord

2. Neuro exam 2 (16 decks)

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