Glioma

Gliomas they are tumors of the brain parenchyma consis�ng of glial cell neuroectodermal epithelial
�ssue include
• astrocytoma
• oligodendroglioma
• ependymoma
• choroid plexus papilloma

1) astrocytoma
it represents 80% of the gliomas
include several different categories of tumors Drive from astrocytes include
• diffuse astrocytoma fibrillary or infiltra�ng
• pilocy�c astrocytoma
• pleomorphic xanthoastrocytoma
• other rare types

1- diffuse fibrillary infiltra�ng astrocytoma

• it accounts for 80% of adult brain tumors
• usually found in hemisphere but may occur in the spinal cord brainstem and cerebellum
• o�en occur in the 4th to 6th decade of life
• clinical picture: headache seizures focal neurological deficit according to the site
• radiological studies show Mass Effect surrounding brain edema the high grade show
abnormal leaky vessels so enhance their contrast
• morphology:
 macroscopic
poorly defined grayish infiltra�ve tumor
range in size from few millimeters to big size replacing the hemisphere
consistency is either firm so� or gela�nous and cys�c degenera�ve changes may be seen
n.b in glioblastoma there is varia�on in the consistency of tumors some areas are firm
white other unsolved others or so� and yellow due to �ssue necrosis and other areas
through cys�c degenera�on and hemorrhage. There is infiltra�on beyond the outer
margin is always present

 microscopic there are four types:

 well differen�ated astrocytoma
 anaplas�c astrocytoma
 glioblastoma
 pleomorphic xanthoastrocytoma

well differen�ated astrocytoma

characterized by
 mild to moderate increase in number of glial cell nuclei
 variable nuclear pleomorphism
 GFAB posi�ve (glial fibrillary acidic protein) astrocy�c cell process that gives the background
fibrillary appearance
  the transi�on between normal and neoplas�c �ssue is less clear

anaplas�c astrocytoma
 become more densely cellular
 more mito�c ac�vity and increase in the mito�c figure
 greater nuclear pleomorphism and nuclear hyperchromatosis

glioblastoma (previously called glioblastoma mul� forms)

similar to anaplas�c type with necrosis and vascular endothelial cell prolifera�on:

 necrosis o�en Serpen�ne patern and occur in areas of hypercellularity necro�c region is
surrounded by highly malignant cell crowded around the necrosis producing astrological patern
called pseudo palisading
 vascular endothelial cell prolifera�on
characterize by tu�s of vascular cell that bulge into vascular lumen,forms a ball like structure
called glomerloid body
 there is vascular endothelial growth factor produced by malignant astrocyte is the cause of this
vascular change

gemistocy�c astrocytoma
it is a term used for tumors in which the malignant astrocytes show esinophilic cell body from which
abundant cell processes arise

n.b gliomatosis cerebri a condi�on where mul�ple regions of the brain and some�mes the en�re brain
are infiltrated by malignant

astrocytes WHO grading system

juvenile pilocy�c 1
well differen�ated astrocytoma grade 2:4
anaplas�c astrocytoma grade 3:4
glioblastoma 4

molecular gene�c
 low grade astrocytoma: among the most common altera�on found in or inac�va�on of B 53
over exhibi�on of PDGF-A gene
 the transi�on to higher grid is associated with addi�onal disturbance of two well known tumor
suppressor gene which is RB and B16

prognosis : survival only 8-10 monthes

Pilocy�c astrocytoma

Criteria
• more in children and young adults
• Rela�vely benign behaviour usually cerebellum floor walls of the third ventricle
• op�c nerve and some�mes cerebral hemisphere

morphology
macro
o�en cys�c with mural nodules in the wall of the cyst
if solid it may be well circumscribed
micro

• tumor is composed of bipolar cells (piloid) with long thin here like process that are GFAP posi�ve
and form dense fibrillary meshwork
• Rosenthal fibers
• Eosinophilic granular bodies
• increase number of blood vessels o�en with thickened wall and vascular prolifera�on and
• necrosis and mitosis are a common
• they have a narrow infiltra�ve border with the surrounding brain

prognosis
• tumours grow very slowly par�cularly in the cerebellum
• recurrence of incompletely restricted lesions associated with cys�c enlargement brothers and
growth of solid board extends into hypothalamic regions Have more difficult clinical course due
to its loca�on due to risky of B 53 muta�ons or other gene�c changes that are found in
infiltra�ve astrocytoma shi� next line next line

Pleomorphic xanthoastrocytoma:
Criteria
most commonly superficial in temporal loop of children and young adult
Clinical picture
seizures and signs of increased intracranial tension
Morphology:
macro
circumscribed no adjacent edema causing scalloping over bound due to superficial posi�on
micro
the tumor consists of neoplas�c bizarre astrocyte which are some�mes liquidized
the degree of nuclear atypia can be extreme and may suggest high grade astrocytoma
abandoned re�culin deposit
rela�ve circumscrip�on chronic inflammatory cell infiltrate
absent necrosis and mito�c ac�vity suggest that diagnosis low grade tumor 2 / 4
prognosis: five years survival rate es�mated at 80% of cases the cruises under methodic ac�vity are
indica�ve of high-grade tumor with more aggressive course
Oligodendroglioma (myelin sheath producing cells)
Criteria
• 5 to 15% of all gliomas
• most common in 4th and 5th decade
• site mostly in cerebral hemisphere with predilec�on for white mater mostly frontal lobes
morphology
macro
well circumscribed gela�nous grey mass o�en with cys�c, focal hemorrhage and calcifica�on

micro
• sheets of regular cell with spherical nuclear containing fine granular chroma�n similar to normal
oligodendrocyte
• surrounded by clear hollow of cytoplasm contained delicate network of anastmosing capillaries
• calcifica�on occurred in 90% of these tumors ranging from microscopic foci to massive deposits
• tumor cell infiltrates cerebral cortex there is forma�on of secondary structures with this
especially with perineural satellitosis
• mito�c ac�vity is very difficult to detect and prolifera�on indices are low
WHO grading: grade 2:4
molecular gene�cs
• the most common gene�c altera�on in oligodendroglioma are lost of heterozygosity for
chromosome 1P and 19 Q in 80% of cases
• addi�onal gene�c altera�on tend to be associated with progression to anaplas�c astrocytoma
• tumors with lots of 1B and 19 Q but without other altera�on have consistent longstanding
response to chemotherapy and radiotherapy
• those with addi�onal gene�c changes have shorter lived response while those without loss of 1P
and 19 Q are resistant to therapy

clinical picture
signs of increase intra cranial tension or focal signs depending on site

prognosis
• have beter prognosis than astrocytoma
• survival with treatment is averaged from 5 to 10 years
• pa�ents with anaplas�c oligodendroglioma have worse prognosis

N.B: the term oligoastrocytoma or anaplas�c oligoastrocytoma referred to neoplasm consistency of

dis�nct region of oligodendroglioma and astrocytoma

Anplas�c oligodendroglioma (WHO grade 3 to 4)

• increased cell density nuclear anaplasia, mito�c ac�vity and necrosis

• these changes can be found in a nodule within an otherwise grade two to four
oligodendroglioma
• there are o�en discrete round cell with cytoplasmic GFAP and nuclei that resemble the other
elements of the tumor
• microgametocytes differ that in astrocytoma in that the lack abandoned processes
ependymoma and related ventricular mass lesion
ependyma is the lining of the fluid filled cavi�es (ventricular system)
criteria
• ependymoma mostly rise next to even ependyma lining ventricle ventricular system including
central canal of spinal cord
• mostly occur in 1st and 2nd decades they occur near 4th ventricle and they cons�tute five to 10%
of primary brain tumor
• in adult spinal cord is the most common loca�on tumours in this site are par�cularly frequent in
the se�ng of neurofibromatosis type 2

morphology
macro
in the 4th ventricle

• they are solid or papillary messes extending from the floor of the ventricle
• o�en demarcated from the adjacent brain than astrocytoma
• near vital pon�ne and medullary nuclear usually make complete resec�on impossible

in intraspinal tumors
sharp demarca�on makes total removal easy

micro
• increased cells with regular round over nuclei with abandoned granular chroma�n
• there is dense fibrillary network between nuclei
• cells may form gland like round elongated structure rosete or canals that resemble the
embryologic epididymal canal with long process extending into the lumen
• vascular pseudorosete: T cell arranged around vessels with intervening zone of thin process
directed to the wall of the vessel
• GFBO expression is found
WHO grading
most tumors are well differen�ated and behave as WHO grade two to four
the anaplas�c ependymoma behave as WHO grade three to four with increased regularity high mito�c
rate, areas of necrosis and decrease ependymal differen�a�on

myxopapillary ependymoma:
• dis�nct but related lesion occurred in filum terminal of spinal cord
• contain papillary element in myxoid background with ependymal cell
• papillary: cuboid cells with clear cytoplasm are arranged around papillary cores contain
connec�ve �ssue and blood vessel
• myxoid area contain neutral and acidic mucopolysaccharide

prognosis
• depend on completeness of surgical resec�on
• if tumors extend to subarachnoid space recurrence is less likely recurrence is likely
molecular gene�cs of ependymoma
• NF 2 gene on chromosome 22 is commonly mutated in spinal cord but not on all other side
• supratentorial region are more likely to show altera�on in chromosome 9

clinical picture
present with hydrocephalus secondary obstruc�on of the 4th ventricle rather than invasion of the
bones of medulla and signs of increased internal tension

prognosis
• poor prognosis despite slow growth and lack of histologic evidence of anaplas�c ac�vity
• CSF discrimina�on is common
• Post. fossa tumor had the worst prognosis with five year survival of roughly 50%
• clinical outcome for resected supratentorial and spinal tumors is beter

subtypes of ependymoma:
1. subependymoma
• solid some�mes calcified slow growing nodule atached to the ventricle lining
• usually asymptoma�c
• o�en found in lateral and forced ventricle
• if sufficiently large or strategically located may cause hydrocephalus

2. choroid plexus papilloma

• site anywhere along choroid plexus
• common in children and most o�en in the lateral ventricle and adult mostly in the
4th ventricle
• clinical picture hydrocephalus due to obstruc�on of the ventricular system by
tumor or overproduc�on of the CSF
• micro papillae having connec�ve �ssue stalks covered with Cupid or column
epithelium

3. colloid cyst of the third ventricle

• non neoplas�c lesion that occur in young adult
• cyst is atached to the roof of 3rd ventricle
• capable of obstruc�ng one or both of the foramina of monro
• clinical picture posi�onal headache