Neoplasms I-III Flashcards
Dx?
- variety of appearances
- inactivation of neurofibromatosis gene Merlin on ch22
- less favorable prognosis
- meningeal-based tumor
hemangiopericytoma
What is a WHO grade II brain lesion?
- infiltrative in nature
- often recur
- tend to progress to higher grade of malignancy
- tx depends on size, type, location, and clinical features
Tell me everything there is to know about anaplastic ependymoma.
- more mitotically active
- childhood (4th ventricle)
- recurs
- WHO grade III
What is Li-Fraumeni Syndrome?
- germ line mutation in p53
- malignant gliomas
Tell me everything there is to know about mixed anaplastic oligoastrocytoma.
- admixed tumor cell population
- increased mitotic activity
- microvascular prolif
- NO necrosis
- LOH 1p, 19q = better prognosis
- WHO grade III
Dx?
- auto dominant
- intra- and extracranial Schwann cell tumors
- optic gliomas
- astrocytomas
- meningiomas
- 17q11.2 encodes neurofibromin protein that normally inhibits RAS
Neurofibromatosis Type 1 (NF1)
Dx?
- usu temporal lobe
- cystic
- well demarcated
- more calcified
- jumbled, abnormal neurons in low grade glial bakground
gangliogliomas
Tell me everything there is to know about Pilocytic Astrocytomas.
- common in childhood
- affects cerebellar hemispheres, optic nerve and chiasm, and hypothalamic region
- well circumscribed, non-infiltrative
- cured by surgical excision alone
- very little tendency to go malignant
- cystic looking, piloid astrocytes, can be calcified, contains mucin and microcysts
- B-raf activation common (good)
- WHO grade I
What kinds of cells give rise to gliomas?
neuroextoderm cells such as glia
Dx?
- arises in white matter
- cerebral hemisphere
- 30-50yo pts
- infiltrative growth pattern
- surgery for debulkment
- mild hypercellularity
- mild nuclear pleomorphism
- irregular astrocyte distribution
diffuse astrocytoma (WHO grade II)
Dx?
- bilateral vestibular schwannomas
- multiple meningiomas
- systemic schwannomas
- systemic liomas
- ependymomas of spinal cord
- chromosome 22q12 encodes Merlin protein that regulates cell receptor signaling
Neurofibromatosis Type 2 (NF2)
What is a WHO grade IV brain lesion?
- cytologically malignant
- mitotically active
- necrosis-prone
- rapid evolution
- fatal outcome
What kinds of cells give rise to meningiomas?
meningeal/mesenchymal cells
Tell me everything there is to know about diffuse astrocytoma.
- arises in white matter
- cerebral hemisphere
- 30-50yo pts
- infiltrative growth pattern
- surgery for debulkment
- mild hypercellularity
- mild nuclear pleomorphism
- irregular astrocyte distribution
- WHO grade II
Dx?
- germ line mutation in p53
- malignant gliomas
Li-Fraumeni Syndrome
Tell me everything there is to know about anaplastic oligodendroglioma.
- (+) enhancement
- round, uniform nuclei w/ scant cytoplasm = “fried egg” appearance
- calcification
- microvascular proliferation
- more mitotic activity
- (+) MIB-1 labeling
- LOH 1p, 19q = good, without = bad
- WHO grade III
Dx?
- admixed tumor cell population
- increased mitotic activity
- microvascular prolif
- NO necrosis
- LOH 1p, 19q = better prognosis
mixed anaplastic oligoastrocytoma (WHO grade III)
What is a WHO grade III brain lesion?
- lesions w/ evidence of malignancy
- nuclear atypica
- brisk mitotic activity
- tx = radiation or chemo
What is Turcot Syndrome?
- medulloblastomas or glioblastomas
- mutation of the APC
Grade the lesion:
- evidence of malignancy
- nuclear atypica
- brisk mitotic activity
- tx = radiation or chemo
WHO grade III
Dx?
- adults
- cerebral hemisphere white matter
- presents with seizures
- calcified
- “chicken wire” vascular pattern
- round, monotonous nuclei + little to no cytoplasm = “fried egg” appearance
oligodendroglioma (WHO grade II)
What are the 5 most common 1a sites that metastasize to the CNS?
- lung
- breast
- melanoma
- kidney
- GI
Tell me everything there is to know about mixed oligoastrocytoma.
- astrocytes and oligodendrocytes admixed in the same tumor
- GFAP (+) on stain
- minimal to absent mitotic activity
- necrosis and microvascular prolif negative
- cell cycle variable but NO G0
- WHO grade II
Tell me everything there is to know about hemangiopericytomas.
- variety of appearances
- inactivation of neurofibromatosis gene Merlin on ch22
- less favorable prognosis
- meningeal-based tumor
Tell me everything there is to know about oligodendroglioma.
- adults
- cerebral hemisphere white matter
- presents with seizures
- calcified
- “chicken wire” vascular pattern
- round, monotonous nuclei + little to no cytoplasm = “fried egg” appearance
- WHO grade II
Grade the lesion:
- infiltrative in nature
- often recur
- tend to progress to higher grade of malignancy
- tx depends on size, type, location, and clinical features
WHO grade II
Dx?
- mutation in PTCH gene
- upregulation of Shh pathways
- medulloblastomas
Gorlin syndrome
Tell me everything there is to know about choroid plexus papillomas.
- always intraventricular locations
- papillary formations
- children =lateral ventricles
- adults = 4th ventricle
- low mitotic rate, mild nuclear atypia
- easily curable with surgical excision
- produces hydrocephalus by blocking CSF flow
- WHO grade I
Dx?
- medulloblastomas or glioblastomas
- mutation of the APC
Turcot Syndrome
How do CNS neoplasms cause death?
- destroy vital brain areas
- by mass effects (tumor + edema = herniation)
Tell me everything there is to know about glioblastoma.
- most malignant
- majority are de novo (primary)
- pts in 50s-60s
- infiltrative
- hemorrhagic
- necrotic
- multifocal origin
- evolve over time
- not surgically curable but done to debulk
- irradiation (temozolomide)
- median survival less than 1 year
- IDH1 and IDH2 mutations = better
Dx?
- peripheral or cranial nerve sheath tumors
- mostly benign but can become neurofibrosarcomas
- esp 8th cranial nerve –> acoustic neuromas proximal
- spinal nerves –> dumbbell shaped tumor
schwannomas
Dx?
- more mitotically active
- childhood (4th ventricle)
- recurs
- WHO grade III
anaplastic ependymoma
What is von Hippel-Lindau (VHL) Syndrome?
- germ line mutation of VHL gene
- downreguated hypoxia-inducible factor 1 (HIF-1)
- overexpression of VEGF and EPO
- polycythemia
- hemangioblastomas
What tumor grade is the most aggressive?
IV (4)
Tell me everything there is to know about Neurofibromatosis Type 2 (NF2).
- bilateral vestibular schwannomas
- multiple meningiomas
- systemic schwannomas
- systemic liomas
- ependymomas of spinal cord
- chromosome 22q12 encodes Merlin protein that regulates cell receptor signaling
Dx?
- always intraventricular locations
- papillary formations
- children =lateral ventricles
- adults = 4th ventricle
- low mitotic rate, mild nuclear atypia
- easily curable with surgical excision
- produces hydrocephalus by blocking CSF flow
choroid plexus papilloma
Dx?
- women > men
- age 30+ (esp in 50s)
- problem only when arises in surgically difficult location
- solitary
- arise from cell called “arachnoid cap”
meningiomas “mostly WHO grade I”
Dx?
- most malignant
- majority are de novo (primary)
- pts in 50s-60s
- infiltrative
- hemorrhagic
- necrotic
- multifocal origin
- evolve over time
- not surgically curable but done to debulk
- irradiation (temozolomide)
- median survival less than 1 year
- IDH1 and IDH2 mutations = better
glioblastoma
Tell me everything there is to know about ependymoma.
- from ependymal cells lining the ventricles
- found in intraventricular locations
- pts 0-20yo –> usually 4th ventricle
- adult pts –> spinal cord
- presents w/ obstructive hydrocephalus
- calcified
- demarcated in spinal cord –> excision
- brainstem location –> no excision (location dictates prognosis)
- perivascular
- pseudorosettes
- WHO grade II
Tell me everything there is to know about anaplastic astrocytoma.
- increased mitotic rate
- MIB-1 nuclear labeling (+)
- no necrosis or microvascular prolif
- crowded cells
- nuclear hyperchromatism and size/shape variation
- prognosis = dependent upon growth rate
- WHO grade III
Dx?
- common in childhood
- affects cerebellar hemispheres, optic nerve and chiasm, and hypothalamic region
- well circumscribed, non-infiltrative
- cured by surgical excision alone
- very little tendency to go malignant
- cystic looking, piloid astrocytes, can be calcified, contains mucin and microcysts
- B-raf activation common (good)
Pilocytic Astrocytoma
Dx?
- auto dominant
- caused by mutation in TSC1 and TSC2 genes that inhibit mTOR (cell size and anabolic growth regulator)
- hamartomas and benign neoplasms
- sub-ependymal giant cell astrocytomas
Tuberous Sclerosis
Tell me everything there is to know about Tuberous Sclerosis.
- auto dominant
- caused by mutation in TSC1 and TSC2 genes that inhibit mTOR (cell size and anabolic growth regulator)
- hamartomas and benign neoplasms
- sub-ependymal giant cell astrocytomas
_____ metastases are typically sharply demarcated, treated with surgical excision, and are commonly found at the grey-white junction.
Intraparenchymal
Dx?
- malignant embryonal tumor of the cerebellum
- occurs in children (3-8yo, esp males)
- spreads via CSF pathways
- most common type of PNET
- presents with increased ICP signs (HA, vomiting, papilledema) and gait, nystagmus, and dysmetria problems
- responsive to aggressive chemo and radiation IF no CSF spread
- patternless sheets of small embryological cells w/ scant cytoplasm (small blue cells)
- Homer Wright rosettes
- problem with Shh pathway
medulloblastoma (WHO grade IV)
Pediatric pts develop about 2/3 of their brain tumors in the _____ rather than the _____ space.
infratentorial; supratentorial
What is the most common type of primary brain tumor?
astrocytomas
Tell me everything there is to know about Neurofibromatosis Type 1 (NF1).
- auto dominant
- intra- and extracranial Schwann cell tumors
- optic gliomas
- astrocytomas
- meningiomas
- 17q11.2 encodes neurofibromin protein that inhibits RAS
Dx?
- germ line mutation of VHL gene
- downreguated hypoxia-inducible factor 1 (HIF-1)
- overexpression of VEGF and EPO
- polycythemia
- hemangioblastomas
von Hippel-Lindau (VHL) Syndrome
Tell me everything there is to know about schwannomas.
- peripheral or cranial nerve sheath tumors
- mostly benign but can become neurofibrosarcomas
- esp 8th cranial nerve –> acoustic neuromas
- proximal spinal nerves –> dumbbell shaped tumors
Grade the lesion:
- cytologically malignant
- mitotically active
- necrosis-prone
- rapid evolution
- fatal outcome
WHO grade IV
Tell me everything there is to know about meningiomas.
- women > men
- age 30+ (esp in 50s)
- problem only when arises in surgically difficult location
- solitary
- arise from cell called “arachnoid cap”
- mostly WHO grade I
Dx?
- increased mitotic rate
- MIB-1 nuclear labeling (+)
- no necrosis or microvascular prolif
- crowded cells
- nuclear hyperchromatism and size/shape variation
- prognosis = depends on growth rate
anaplastic astrocytoma (WHO grade III)
Tell me everything there is to know about medulloblastoma.
- malignant embryonal tumor of the cerebellum
- occurs in children (3-8yo, esp males)
- spreads via CSF pathways
- most common type of PNET
- presents with increased ICP signs (HA, vomiting, papilledema) and gait, nystagmus, and dysmetria problems
- responsive to aggressive chemo and radiation IF no CSF spread
- patternless sheets of small embryological cells w/ scant cytoplasm (small blue cells)
- Homer Wright rosettes
- problem with Shh pathway
- WHO grade IV
What is a WHO grade I brain lesion?
- tumors w/ low proliferative potential
- possible cure by surgical resection alone
Grade the lesion:
- tumors w/ low proliferative potential
- possible cure by surgical resection alone
WHO grade I
Dx?
- astrocytes and oligodendrocytes admixed in the same tumor
- GFAP (+) on stain
- minimal to absent mitotic activity
- necrosis and microvascular prolif negative
- cell cycle variable but NO G0
mixed oligoastrocytoma (WHO grade II)
Dx?
- from ependymal cells lining the ventricles
- found in intraventricular locations
- pts 0-20yo –> usually 4th ventricle
- adult pts –> spinal cord
- presents w/ obstructive hydrocephalus
- calcified
- well demarcated in spinal cord –> excision
- brainstem location –> no excision (location dictates prognosis)
- perivascular pseudorosettes
ependymoma (WHO grade II)
Tell me everything there is to know about Gangliogliomas.
- usu temporal lobe
- cystic
- well demarcated
- more calcified
- jumbled, abnormal neurons in low grade glial background
- WHO grade I
What is Gorlin syndrome?
- mutation in PTCH gene
- upregulation of Shh pathways
- medulloblastomas
Dx?
- (+) enhancement
- round, uniform nuclei w/ scant cytoplasm = “fried egg” appearance
- calcification
- microvascular proliferation
- more mitotic activity
- (+) MIB-1 labeling
- LOH 1p, 19q = good, without = bad
anaplastic oligodendroglioma (WHO grade III)
Name 2 different cell classes that CNS neoplasms can arise from and what class of tumors they become.
- cells within the neural tube –> primary/intrinsic brain tumors
- cells from supporting structures —> extrinsic brain tumors
