Non-Glial Tumors
- Central
neurocytoma
- Septum
pellucidum/foramen of monro
- Young
adult
- Circumscribed,
lobulated, enhancing, calcified, rare mitosis, frequent cyst and
necrosis, occasional hemorrhage
- Synaptophysin+
- monotonous
hypercellularity like oligodendroglioma
- Ependymoma
- Peak
incidence is 1st and 2nd decades
- Biphasic
1 year, 35 years; M>F
- Supratentorial
in children, fourth ventricle in adults, also found in spinal cord
- Arises
from floor of 4th ventricle and may extend into the CP angle
or cisterna magna
- 50%
calcify
- cellular,
papillary, myxopapillary (sacral), clear cell variants
- cell
population monomorphic with rosettes, pseudorosettes (around blood
vessel), blepharoplasts
- GFAP+,
PTAH+
- Look
similar to medulloblastoma but smaller nuclei, fewer mitoses, GFAP+ and
synaptophysin- (medulloblastoma GFAP- synaptophysin+)
- Radiology:
Multiobulated irregularly enhancing mass within 4th ventricle,
50% have calcifications
- Treatment:
Surgical excision and XRT
- May
metastasize through the CSF
- not much
known about optimal treatment
- in
children surgery, radiation, chemotherapy
- in
adults surgery and craniospinal irradiation
- Prognosis:
2-year mean survival
- Subependymoma
- 40-60
years, M>F
- floor of
fourth ventricle or septum pellucidum
- hypocellular
nests of cells separated by glial fibrils
- uniform
cells, microcysts, calcification, vascular hyalinization, hemosiderin,
mitosis none of these have prognostic value
- no
necrosis, rosettes, or seeding
- Choroid
plexus papilloma one of most common tumors <2 years
- 1% of
brain tumors
- Peak
incidence is in the 1st decade
- lateral
ventricle in childhood (L>R), fourth ventricle in adults, 10% in 3rd
ventricle and CPA, 4% bilateral
- no cilia
except in very young children
- 25%
calcify
- Hydrocephalus
is often present at presentation
- Radiology:
High density, speckled intraventricular lesion with calcifications and
bright enhancement with contrast
- Treatment:
Surgical excision
- Prognosis:
Generally benign with long-term survival. 20% have malignant degeneration
with 2-3 years median survival.cells in single layer (helps differentiate
from papillary ependymoma which is multilayered)
- stains
for transthyretin, vimentin, keratin, S100
- choroid
plexus carcinoma 15% of choroids plexus tumors
- peak
age 2 years
- differentiate
from metastasis by immunochemistry (EMA+, S100-)
- Primitive
Neuroectodermal Tumor (PNET)
- Medulloblastoma
- Usually
near 4th ventricle in children
- Peak
incidence is in 1st decade with male predominance; a second
peak occurs in adults 18-30 years
- Most
common solid tumor in children, second most common childhood CNS tumor
- Arises
in vermis of cerebellum
- Associated
with basal cell nevus syndrome
- Homer-Wright
Rosettes around central granulofibrillar material with radially
arranged nuclei
- Linear
Indian file and round island of cells
- 50%
metastasize 2/3 CNS, rest to bone
- 20-50%
disseminated at diagnosis
- Radiology:
Nonhomogenously enhancing midline cerebellar mass, only 10% calcify
- Treatment:
Surgical excision, XRT, chemotherapy
- May metastasize
through the CSF
- Prognosis:
5-year mean survival; some long-term survivors
- Retinoblastoma most common malignant solid
tumor in children; most less than 5 years of age
- Flexner-Wintersteiner
rosettes columnar, small lumen, like cones of retina (also found in
pinealoblastoma)
- Central
neuroblastoma
- supratentorial,
hemispheric, circumscribed
- usually
less than 5 years
- hemorrhagic
with necrosis and cyst
- Neuroblastoma 3rd most common
tumor in children after leukemia/brain tumors
- 2% are
cerebral
- also
found adrenal
- common
spinal epidural metastasis in children
- Medulloepithelioma tumor of ventricular matrix
cells; most primitive of all brain tumors
- Meningioma 15% of intracranial tumors
- Rarely
occur before 3rd decade, female predominance
- Are associated
with neurofibromatosis and breast Ca
- Locations:
Parasagittal (21%), convexity (15%), tuberculum sella (13%), sphenoid
wing (12%), olfactory groove (10%), falx (8%), intraventricular (4%),
tentorium (4%), middle fossa (3%), orbital (1%), spinal (1%)
- Presentation:
HA, seizure, focal neurologic deficit, slowly declining mental status
- 2/3 of
these tumors have monosomy 22
- more
often osteoblastic than lytic
- cell of
origin arachnoid cap cell
- 50% are
parasagittal or convexity; other locations: tuberculum > olfactory
groove > foramen magnum > optic nerve > tentorial > choroid
plexus (left) > thoracic spine
- 9%
multiple
- can be
atypical (6%, increased cellularity, necrosis, mitosis) or anaplastic
(2%, invasion and metastasis)
- sunburst
dural feeders, basophilic psamoma bodies, whorls
- stain
for vimentin and EMA (mesenchymal and epithelial origin)
- Types:
- Meningothelial (syncitial) whorls,
lobules, few psammoma bodies
- Fibroblastic sheets of cells
- Transitional
- Psammomatous
- Angioblastic
- Radiology:
Dural based usually hyperdense homogenously enhancing sharply demarcated
mass, ± associated cerebral edema
- Treatment:
Surgical excision (XRT only if malignant, recurrent, or unable to be
completely resected)
- Prognosis:
Many long-term survivorsHormone receptors (enlarge during
pregnancy/breast cancer) estrogen, progesterone, peptide, amine,
cortisol, somatostatin, CCK
- Hemangioblastoma
- 2% of
intracranial tumors, 10% of posterior fossa tumors (also found in spine)
- Most
common primary posterior fossa tumor in adults
- Associated
with von Hippel-Lindau syndrome; 25% with this tumor have other features
of von Hippel-Lindau
- 20-40
years old, M>F
- 50%
cystic; increased reticulin, no mitosis, rare calcium/necrosis
- 80%
sporadic, rest vHL (multiple)
- hypoplastic
endothelial cells, pericytes, stromal cells with lipid vacuoles
- 10% have
polycythemia
- May be
multiple
- Radiology:
Hemispheric cystic lesion with region of nodular enhancement
- Angiography
is necessary given tumors vascularity
- Treatment:
Surgical excision
- Prognosis:
Many long-term survivors
- Recurrent
disease in those with von Hippel-Lindau
- Craniopharyngioma
- 2-5% of
intracranial tumors
- Most
common supratentorial tumor in children, but also seen in adult
population
- 2 peaks:
0-20 years, 50 years; M>F
- 60% suprasellar
or infrasellar; rarely sellar, CPA, pineal, nasopharyngeal
- from
squamous cells of rathke cleft
- cyst
with nodule filled with machine oil fluid and cholesterol crystals with
granulomatous reaction
- most
calcify (100% children, 50% adult)
- sharp
irregular margins with surrounding gliosis
- adamantinomatous
rests of epithelial cells surrounded by layer of columnar basal cells
separated by myxoid stroma of loose stellate cells; whorles and wet
keratinized nodules also present
- Presentation: Visual abnormalities,
partial hypopituitarism, small stature, obstructive hydrocephalus
- Radiology: solid/cystic
suprasellar lesion with variable enhancement and calcification
- Rx: Surgical excision ± XRT
- Prognosis: Lesion is benign, but
difficult to completely resect given location and its tendency to adhere
to the surrounding structures
- Generally
recurs, but may survive for years
- Hemangiopericytoma
- 2% of
meningioma
- 40-50
years, M>F
- usually
supratentorial
- similar
prognosis to anaplastic meningiomas (30% metastasize, 70% recur); no
psammoma bodies, no whorles
- intracranial
blood supply (unlike mengiomas)
- dense
cellularity, frequent mitosis, increased reticulin
- lobules
around staghorn vascular channels
- vimentin+,
EMA-
- Lipoma 0.2% of intracranial tumors
- M=F,
majority in midline (corpus callosum, quadrigeminal plate, third
ventricle, CP angle, sylvian fissure
- 50%
associated with brain malformation
- maldifferentiation
of meninx primitiva (mesenchymal derivative of neural crest)
- tubulonodular
anterior corpus callosum with frontal lobe abnormalities and callosal
agenesis
- curvilinear
in splenium, corpus callosum normal
- demonstrates
peripheral calcification (visible on plain film)
- Paraganglioma
- 30-50
years old, F>M
- glomus
jugulare arises from paraganglia in adventitia of dome of jugular bulb
- produce
catacholamines, very vascular
- Carotid
body tumor
- Painless
mass below jaw at bifurcation of carotid (like salivary tumor, branchial cleft
cyst)
- Neurosecretory
granules and catecholamines
- 5%
bilateral, 5% malignant, often family history
- Esthesioneuroblastoma
- 50 years
- high in
nasal cavity
- neurosecretory
receptor cells or basal cells
- small
round blue cells
- Orbital
Masses
- Symptoms:
Proptosis, ± pain, ± orbital pulsations and
vascular injection, decreasing visual acuity
- Diagnosis:
Disc pallor, MRI imaging
- Etiology:
- Neoplastic:
meningioma (40%), Hemangioma (10%), Glioma (5%), Neurofibroma,
Epidermoid, Lipoma
- Non-neoplastic:
Cellulitis, mucocele, cavernous sinus thrombosis, carotid-cavernous
sinus fistula, histiocytosis, arteriovenous malformation,
thyrotoxicosis, pseudotumor
- Treatment:
Surgical excision vs. XRT