Malignant Flashcards
(41 cards)
Most common primary CNS malignancy in adults
Glioblastoma, IDH wild type (CNS WHO grade 4)
Also H3-wildtype
What are the secondary structures of scherer
Perineuronal satellitosis
Subpial spread
Perivascular accumulation
Growth along white matter tracts
To diagnose glioblastoma you should have at least one of the following
Microvascular proliferation
Pseudopalisading necrosis
Polysomy 7 and monosomy 10 (+7/-10)
EGFR gene amplification
TERT promoter mutation
This is a diffusely infiltrating astrocytic neoplasm with a mutation, and either I DH1 or I DH2 and intact chromosomes 1p and 19q
IHC
Astrocytoma, IDH mutant (CNS WHO grade 2-4)
IDH1 R132H mutation
ATRX loss and TP53 mutant
Differentiate grades 2 to 4 of astrocytoma
G2: lack significant mitotic activity. No CDKN2A/B homozygous deletion
G3: brisk mitotic activity. No CDKN2A/B homozygous deletion
G4: at least has 1 of the ff:
MVP
Pseudopalisading necrosis
CDKN2A/B homozygous deletion
This is a diffusely infiltrating glial neoplasm with monomorphic round nuclei with nuclear halos. They’re also microcalcifications and cystic degeneration. Also seen is chicken wire vasculature
IHC
Grades
Oligodendroglioma, IDH- mutant and 1p/19q co-deleted (CNS WHO Grade 2-3)
Wild type TP53 and ATRX
+ Olig2, variable GFAP
G2: low mitotic activity. No MVP or necrosis
G3: at least one of the ff: brisk mitotic activity, MVP, pseudopalisading necrosis
A tumor in the brainsten of a five year old male was seen. It consists of infiltrating tumor cells with small and monomorphic nuclei with some mitoses.
(+) H3K27M, Olig2, GFAP
Dx
Diffuse midline glioma, H3K27-altered (CNS WHO Grade 4)
Poor prognosis
Sometimes with tp53 and atrx mutation
It is the most common glioma in children
And can be seen in NF1 syndrome
Dx
Morpho
Molecular
Pilocytic astrocytoma (CNS, WHO, grade 1)
It is biphasic with compact zone containing bipolar piloid cells and Rosenthal fibers, and a loose zone with microcysts, myxoid background, and eosinophilic granular bodies (EGBs)
May have MVP but not aggressive
+ gfap, olig2; low ki67
KIAA1549-BRAF fusion MC
Astrocytic tumor with large, pleomorphic, and frequently multinucleated spindled and lipidized cells. Dense reticulin network, EGBs and perivascular lymphocytes.
Dx
Mc location molecular
Grades
Pleomorphic xanthoastrocytoma (CNS WHO Grade 2-3)
Temporal lobe
BRAF V600E and CDKN2A/B homozygous deletion
G2:<5 mitoses/10hpfs
G3: >5
This is a benign, slow growing, well circumscribed tumor, composed of large gemistocytic astrocytes with abundant pink glassy cytoplasm. Frequently has giant ganglion like cells with prominent nucleoli.
Diagnosis
Most common location
Association with syndromes
Subependymal giant cell astrocytoma (Grade 1)
Lateral ventricles
Tuberous sclerosis
Good prognosis
This is a circumscribed glial neoplasm with around, cuboidal, or columnar cells invariably papillary, or very vascular growth patterns with hyalinized blood vessels
+ GFAP, Olig2, EMA (diffuse, membranous, dor-like or ring-like)
Dx
Molecular
Astroblastoma, MN1-altered
MN1 alteration, MC fusion: MN1-Abend2 fusion
This is a well circumscribed tumor was clusters and cords of epithelial neoplastic glial cells. It is solid with variably mucinous stroma.
+TTF1, GFAP, CD34
Dx
Mc location
Molecular
Chordoid glioma (grade 2)
Third ventricle
PRKCA D463H mutation
Tumor composed of sheets of uniform, round cells with salt and pepper, chromatin, and perinuclear, halos and calcifications
+ synaptophysin
Central neurocytoma (grade 2)
Mc in lateral ventricles
It is a circumscribed glioma composed of uniform, small cells with round nuclei and speckled chromatin any Fibrillary matrix. There are perivascular pseudorosettes.
+ GFAP, EMA (perinuclear dot-like pattern)
Dx
Grade
Ependymoma (grade 2-3)
Grade based on anatomic location, histomorphology and molecular features
Type of ependymoma seen in NF2
Spinal ependymoma
Loss of chromosome 22
No MYCN amplification
(The other spinal ependymoma is MYCN-amplified and has poor prognosis)
CNS tumor with clusters of mostly nland cells embedded in abundant February matrix. There is frequent microcystic change.
+ GFAP and EMA (focal dot like pattern)
Subependymoma (grade 1)
4th and lateral ventricles
This is a tumor with delicate fiber, vascular fronds, covered by single layer of cuboidal to columnar epithelium. Mitosis is <2/10hpfs
+ CK7/-CK20
+S100
Choroid plexus papilloma (grade 1)
MC in lateral ventricles
Atypical CPP G2: >/=2/10hpfs mitosis, nuclear pleomorphism, solid growth, necrosis
Choroid plexus carcinoma, G3: at least 4 of the ff: >5/10hpf mitosis, increased cellular density, nuclear pleomorphism, blurring of papillary pttern with sheets, necrosis; assoc with Li-Frauneni syndrome (tp53 mutations)
CNS tumor with densely packed small round blue cells and homer-wright rosettes with arrangement in patallel rows.
Reticulin: not significant
Dx
Molecular
Classic medulloblastoma
WNT-activated- childhood, APC mutation
Non-WNT/Non-SHH group 3- infancy/children
Non-WNT/Non-SHH group 4 (MC) - all age groups
CNS tumor with pale nodules surrounded by densely, packed small round blue cells
pale nodules: low ki67, express neuronal markers
Internodular dark zones: high ki67, low expression of neuronal markers, reticulin-rich
Dx
Molecular
Desmoplastic/nodular medulloblastoma
SHH-activated
Tp53 wildtype
(Subgroups SHH1-4)
CNS tumor with lobular architecture with elongated reticulin-free zones containing small neurocytic cells with a fibrillary bg
High ki67
Medulloblastoma with a extensive nodularity (MBEN)
SHH-activated
TP53- wildtype
Subgroups SHH1-4
Subtype of Medulloblastoma with Marked nuclear pleomorphism and high mitotic index and numerous apoptotic bodies. Large cells have prominent nucleoli
Dx
Molecular
Large cell/anaplastic medulloblastoma
SHH activated
TP53 mutant
Subgroup SHH-3
Medulloblastoma
Grade
Location
Ihc
Prognosis
Grade 4
4th ventricle or cerebellar parenchyma
Nonspecific neural markers
SHH-activated: +GAB1, lateral cerebellar hemisphere almost always this type
WNT-activated: +nuclear beta catenin
Desmoplasti and mben: +NeuN
Tp53 mutant: strong Tp53
Classic has excellent prognosis
CNS neoplasm with a variable number of rhabdoid cells with eccentric nuclei, the vesicular chromatin, prominent nucleoli, abundant eosinophilic cytoplasm, numerous mitosis and geographic necrosis
Dx
Grade
Ihc
Atypical teratoid/rhabdoid tumor (grade 4)
(-) SMARCB1
Sheet-like growth of uniform, small, mature cells with round nuclei and fine chromatin. There are also large rosettes.
+ synaptophysin, NSE, neurofilament
Ki67 <1%
Pineocytoma (grade 1)
Pineal parenchymal tumor of intermediate differentiation (PPTID, grade 2-3): increased cellularity, ki67 >5%
Pineoblastoma (grade 4)
Highly celular, patternless pattern of smal immature neuroepithelial cells
Ki67 >20%
Necrosis common
