Tumors of CNS Flashcards
What is the most common type of brain tumor in adults?
Mets same frequency as Primary (relatively uncommon)
Above Tentorium (cerebrum_
Mets go to Infratentorium
What is the most common type of brain tumor in adults?
Mets
What is the most common type of brain tumor in children?
Primary brain tumor (below tentorium=cerebellum/brain stem)
What is the most common type of brain tumor in children?
Primary brain tumor (below tentorium=cerebellum/brain stem)
What type of tumor do adults get more commonly?
Fibrillary astrocytoma and Glioblastoma
Higher grade
What type of tumors do children get more commonly?
Pilocytic astrocytoma (low grade) Medulloblastoma (embryonal)
What type of spinal cord tumor do adults get more?
Meningioma/schwannoma
What type of spinal cord tumor do children get more common?
Astrocytoma/ependyoma (adult ones are rare)
What makes CNS neoplasma special about histology benign apearance?
Although look benign mass effect can cause major problems makes them malignant fasion
Do primary CNS neoplasms metastasize?
Rarely
What are the most common types of neoplasms to metastasize to CNS?
Lung, Breast, Colon
Choriocarcinoma/melanoma are rare but very frequently go to CNS
What are tumors of glial origin?
Astrocytoma
Oligos
Ependymal
Where are astrocytomas found?
Supporting cells of brain, maintain neurons, immuno surveilance, neurotransmitter clearance, neuropil repair
GFAP positive (glial fibrillary acidic protein)
Where are oligodendrocytes found?
Myelinating cells of CNS
GFAP positive
Where are ependymal cells found?
Ventricular system of CNS
Ventricles, foramens
GFAP positive
What is the most common glial tumor?
Astrocytomas
Next is oligo
Next is epedyoma
What is astrocytoma WHO I?
Pilocystic astrocytoma
FOUND IN CHILDREN
INFRATENTORIAL
Imaging: Cystic Cerebellum mass with ENHANCING MURAL NODULE
- Outside cerebellum rare and are not cystic
Pathology: Densely fibrillated tumor
- Areas of microcytic/fibrillar: Biphasic
- ROSENTHAL FIBERS (eosinophilc granular bodies)
- Does not progress to increased grade
Treatment: Complete resection
What is Astrocytoma WHOII?
FIbrillary Astrocytoma
- Occurs in Cerebral Hemispheres (adults)
- Occurs in Pons (children
Imaging: Infiltrative
- DO not have ENHANCING
- CAN PROGRESS to WHO III, IV
What is Astrocytoma WHOIII?
Anaplastic astrocytoma
Major feature is MITOSES, increased cellularity, atypia
Imaging
What is Astrocytoma WHOIII?
Anaplastic astrocytoma
Major feature is MITOSES, increased cellularity, atypia
Imaging: May be enhancing
What is a glioblastoma?
Astrocytoma WHO IV
Imaging: RING ENHANCING MASS, craggy appearance
-The ring is blood vessels
DDX: ABSCESS, METS, MS, Lymphoma
Histology: Very cellular, pleomorphic with palisading necrosis/glomeruloid vessels (multiple layers)
Two age peaks: Young adults and Older individuals
- Young as a result of astrocytoma progression
- Old is de novo GBM: VERY POOR Prognosis
What is are the mutations associated with de novo GBM?
EGFR amp
PTEN mutation
What are the mutations associated with low grade astrocytoma prgoression?
TP53 mutation
What are common features of GBM?
LOH 10q
p16INK41 deletion
Where are oligodendrocytes found?
Myelinating cells of CNS
GFAP NEGATIVE
What are the two grades of oligos?
WHO II, WHO III
What is a oligodendroglioma?
WHO II
Imagin: May or may not enhance
Path:
ROUND CELLS with pale cytoplasm: FRIED EGG
-Thin walled microvas in background
- Have microcalcs
What is anaplastic oligodendroglioma?
WHO III
Imaging : same as WHO II
Path: More pleomorphism but have FRIED EGG
- More MITS, and NECROSIS
What is the cytogenic assessment of oligodendros?
1p and 19q deletions
- Important response to therapy
- Have a good survivalability with PVU (procarbazine, vincristine, CCNU)
What are ependyomas?
GFAP positive
Only WHO II, WHO III
What are ependyomas?
WHO II
Imaging may or may not enhance
Path: Perivascular
Fibrillar nuclear free zone, surrounding aggregates of uniform nuclei
-PERIVASCULAR PSEUDOROSETTES
What is a anaplastic ependyoma?
WHO III
Same as WHO II but has more pleomorphism and MITS
OCCUR in SPINAL CORD MORE COMMONLY IN ADULTS
What are tumors of the DURA and ARACHNOID?
Meningioma
Arise from arachnoid are are attached to teh dura
RARE IN CHILDREN
HERALD NEUROFIBROMATOSIS
KIDS: 4th ventricle
ADULTS: lateral vents
More common in women (progresterone positive)
Can be small but lethal due to location (foramen magnum is lethal
IMAGING: Enhance uniformly and brightly, DURAL TAIL
Boat shaped, oval nuclei with eos cytoplasma
Meningothelial whorls
Psammoma bodies
What is WHOI meningioma?
Few mits, well circumscribed, DO NOT INFILTRATE
What is WHO II meningioma?
4-20 mts/HPF, brain INVASION Increased ugliness - Increased cellularity - Small cell - Macronucleoli -Sheeting -SPontaneous
Who is WHO III meningionma?
ANAPLASTIC
>20mits/HPF,
Who is WHO III meningionma?
ANAPLASTIC
>20mits/HPF
Papillary/rhabdoid morphology
Might have MONOSOMY 9p21
What are embryonal tumors?
CHILDREN/ YOUNG ADULT
Small round blue cell tumor (sheets of small cells with hyperchromatic nuclei)
SEED CSF
NEUROBLASTOMa Rare
Medulloblastoma: External granular layer of Cerebellar cortex, VERMIS
What is a medulloblastoma?
MOST COMMON Embryomal tumor
- Forms from granular layer of cerebellum cortex
- Should disappear, usually in VERMIS
Morph variants:
- Classical
- Nodular/desmoplastic
- Medullo with extensive nodularity
- Anaplastic/large cell variant (older children aggressive)
Treatment: Complete resection
Cytogenetics: 17p loss - PTCH, SMOH, SONIC HH -WNT - Gain 1q, 2p, 4p, 7, 19 Loss: 10,11
- nmyc: Cellular anaplasia
- LOH 9q (gorlins)
- APC gene: DNA methylation
Cerebral Neuroblastoma?
RARE but BAD Loss of 3p Loss of Chr 10 P53 mutation rare Poor prognosis overall
What are chorid plexus tumors?
PAPILLOM>CARCINOMA
- KIDS lateral Vent
- Adults: 4th vent
Occasionally causes hydrocephalus due to CSF production
What is a suprasellar mass that presents with visual, endocrine, hypothalamic syndromes?
Craniopharyngioma
From remnants of Rathekye’s pouch (hypopharynx
Wet keratin epithelium (adamantinomatus/squamous)
Calficied to help make diagnosis
Can be removed unless involves optic chiasm, parenchyma of hypothalaus, floor of 3rd ventricle
What is a suprasellar mass that presents with visual, endocrine, hypothalamic syndromes?
Craniopharyngioma
From remnants of Rathekye’s pouch (hypopharynx
Wet keratin epithelium (adamantinomatus/squamous)
Calficied to help make diagnosis
Can be removed unless involves optic chiasm, parenchyma of hypothalaus, floor of 3rd ventricle
What are the most common tumors in adults?
Glioblastoma/fibrillary astrocytoma
What is the most common tumor in children?
Pilocystic astrocytoma/medulloblastoma
What are signs of supratentorial mass?
HA, Seizures, Increased ICP
What are some infratentorial signs?
Raised ICP, Cranial Nerve defects, Cerebellar signs
What are some spinal cord signs?
Pain
MOtor deficit/Sensory deficit
Bladder/bowel problems
