Académique Documents
Professionnel Documents
Culture Documents
|
IMA - sporadic
Immunosupression -
º
V
º
º
V
º
Astrocytes Astrocytomas
Oligodendrocytes Oligodendrogliomas
Ependymal cells Ependymomas
Different types of glia Mixed gliomas (oligoastrocytomas)
|
!
"!
V
#
V
|
p
p
$
%
igh-grade gliomas
ihighly-vascular tumors
itendency to infiltrate
iextensive areas of necrosis and hypoxia
ibreakdown of the blood-brain barrier
ialmost always recur even after complete surgical excision
Low-grade gliomas
igrow slowly
iover many years
ifollowed without treatment unless they grow and cause
symptoms
depends on - location,
- cell type
- grade of malignancy
- hemotherapy
iGliomas cannot be cured
igh-grade gliomas
ioor prognosis
i50% - 1 year after diagnosis
i25% - 2 years after diagnosis
ianaplastic astrocytoma survive about three years
iGlioblastoma multiforme has a worse prognosis
!V
&
'
i
p p
p
p
|
º
a
idepends highly on the location
iasymptomatic condition
iseizure, nausea and vomiting,
iheadache, and hemiparesis
iprogressive
memory, personality, or neurological deficit
due to temporal and frontal lobe involvement
è
iM I ring-enhancing lesions
iDefinitive diagnosis of a suspected GBM on T or M I
stereotactic biopsy or
craniotomy with tumor resection
Sagittal M I
with contrast
glioblastoma
WO grade IV
in a 15-year-old boy
ipresence of small areas of necrotizing tissue
that is surrounded by anaplastic cells
(pseudopalisading necrosis)
idifferentiates Grade 3 astrocytomas
presence of hyperplastic blood vessels
isecondary GBM
degeneration of lower grade gliomas
more common in younger patients
iIn the cerebral white matter, grow quickly
i
!"#
iº|"
i
i
very difficult
iThe tumor cells are very resistant to chemotherapy
and other conventional therapies
iThe brain is susceptible to damage due to therapy
iThe brain has a very limited capacity to repair itself
iMany drugs cannot cross the blood brain barrier
to act on the tumor
iAnticonvulsants
iorticosteroids - reduce peritumoral edema
ito improve quality of life
ito achieve a longer survival time
a
i1011 cells reduced to 109 cells
iThe greater the extent of tumor removal,
the longer the survival time
Y
iradiotherapy after surgery
reduce the tumor size to 107 cells
iA total radiation dose of 60±65 Gy - optimal
istandard of care for glioblastoma is
chemotherapy during and after radiotherapy
ichemotherapy after surgery and radiotherapy
reduce the tumor size to 106 cells
iTemozolomide
during radiotherapy
for six months post radiotherapy
º
$ $
$
$
characteristic À -like cells, with clear cytoplasm and
well-defined cell borders. E stain.
î î
%&
%
iIncurable
islowly growing with prolonged survival
imedian survival times
-11.6 years for grade II
-3.5 years for grade III
ivery high (almost uniform) rate of recurrence
igradually increase in grade over time
p
- Temozolomide
standard dosing schedule
5 consecutive days of daily dosing
during 28 day cycles
iBecause of their diffusely infiltrating nature,
oligodendrogliomas cannot be completely resected
inot curable by surgical excision
i
i
"
ia tumor that arises from the ependyma,
a tissue of the central nervous system
ilocation in children - intracranial
in adults - spinal
common intracrania location
- fourth ventricle
can occur in - pelvic cavity
iSyringomyelia can be caused by an ependymona
iEpendymomas are also seen with Neurofibromatosis Type
II
i
))!
! $"
p
iwell-differentiated ependymomas
radiation therapy only
iother ependymomas
total surgical removal is the preferred treatment
cannot be totally removed - require radiation therapy
imalignant (anaplastic) varieties of this tumor
imalignant ependymoma
iEpendymoblastoma - treated similarly to medulloblastoma
combination of radiation therapy and chemotherapy
iin infants and children younger than 5 years of age,
may spread through the cerebrospinal fluid
usually require radiation therapy
(
ia variant of the ependymoma
iarise in the fourth ventricle
in the septum pellucidum
cervical spinal cord
iover 40 years of age
imen > women
irare,
islow-growing,
ihistologically benign intracranial tumor
icommonly located
in the ventricular system of the choroid plexus
D *
i0.4-0.6% of all intracranial neoplasms
imost commonly affects young children under the age of 5
imean patient age of 5.2 year
ibenign tumors
iusually cured by surgery
imalignant progression has been rarely reported
arise from specialized cells of pineal gland -ineocytes
ineocytoma - also known as a pinealocytoma
- benign, slowly-growing tumor
- low grade tumor
- well-differentiated
- areas of neutropil + tumor cells
with small round nuclei
- no mitoses or necrosis
ineoblastoma - high grade tumor
- densely packed small cells
- necrosis and mitoses
- spread through SF
- more common in children
- may occur in etinoblastoma patients
))! ! $"
)
*
%
%
*
+
u
º
º
V (
V (
ineuroendocrine tumor,
arising from any neural crest element
of the sympathetic nervous system
+,-.
º
Ú Ú
+,/+ º
!
+/0+
,
-
+/+0 .
,
(
Ú 12
Ú
German physician
udolf Virchow
the first to describe an abdominal tumor
in a child as a "glioma"
"
))!
|
'
-
-
#
º
º
" ! $"
-
#
#
'
#
$ 3
-
#%'
#%'
$
usually confirmed by a surgical pathologist
iclinical presentation,
imicroscopic findings,
iother laboratory tests
a
iurine or blood
ielevated levels of catecholamines or its metabolites
- dopamine,
- homovanillic acid (VA),
- vanillylmandelic acid (VMA)
i90% of cases of neuroblastoma
imIBG scan (meta-iodobenzylguanidine)
imIBG-avid
i90 to 95% of all neuroblastomas
à
imIBG is taken up by sympathetic neurons
functioning analog of neurotransmitter norepinephrine
iWhen it is radio-ionated with I-131 or I-123
(radioactive iodine isotopes)
very good radiopharmaceutical for diagnosis and
monitoring of response to treatment for this disease
ihalf-life of I-123 - 13 hours,
preferred isotope for imaging sensitivity and quality.
ihalf-life of I-131 - 8 days
higher doses is an effective therapy as targeted
radiation
against relapsed and refractory neuroblastoma
microscopic view of
stroma-rich ganglioneuroblastoma
'
#(
-
'
#'î
º !
(
iMolecular genetics
iloss of genetic information on the
distal part of chromosome 17, distal to the ½ gene
ineoplastic transformation of the undifferentiated cerebellar cells
½
½
î
3½
½
- facial sensory loss or motor weakness
iLate - decerebrate attacks
isolid
ipink-gray in color
iwell circumscribed
ivery cellular
imany mitoses
ilittle cytoplasm
ihas tendency to form clusters and rosettes
maximal resection of the tumor
to increase the disease-free survival
to the entire neuraxis
- chemotherapy
Increased I - corticosteroids or
- ventriculoperitoneal shunt
combination therapy
5 year survival in > 80% of cases
better prognosis
presence of desmoplastic features
- connective tissue formation
poor prognosis
- less than 3 years old
- inadequate degree of resection
- any SF, spinal, supratentorial or systemic spread
WO classification
isecond most common primary tumor
of the central nervous system
iusually - benign
- can be malignant
imost - sporadic
isome - familial
irisk factor - radiation to the scalp
igenetic mutations - inactivation mutations
in the neurofibromatosis 2 gene (merlin)
on chromosome 22q
Small tumors (e.g., < 2.0 cm)
- usually incidental findings at autopsy
- without having caused symptoms
Larger tumors
- symptoms depending on the size and location
?î
? eventually occurs
but is less frequent than in gliomas
D î
î
î î
arise from arachnoidal cells
most of which are near the vicinity of the venous sinuses
(site of greatest prevalence for meningioma formation)
|
p
î
pî
##%
##%
p
î
'%'
%
÷
Transarterial embolization
standard preoperative procedure in the preoperative
management
-
!
V$
V
D!
1 "!
#"
! $
V
2
! $"
))!
!""
V
º
º
º
p
))! !""V
V
Galactorrhoea
Amenorrhoea (¶,2¶)
)
º)
Type of Secretion Staining athology %
adenoma
½ î (G) acidophilic Acromegaly 20 %
(gigantism)
½ î (L) basophilic 1- 2 %
(FS)
î ½ î / prolactin acidophilic galactorrhea, hypogonadism, 40 %
½
î
amenorrhea,infertility,impotence
º
- rolactin
- high rolactin level - rolactinoma
- preclude the need for surgery
(ituitary stalk compression -
moderately elevate prolactin
level)
AIM
- to alleviate mass effect
- restore or replace endocrine function
- prevent recurrence
lose co-operation of - Neurosurgeon
- Endocrinologist
rolactinoma
- initial treatment - medically - Dopamine agonist
- abergoline
- Bromocryptine
Growth hormone secreting tumour
- medically - Somatostatin
analogues
- Octreotide
- Dopamine agonist
Transsphenoidal Surgery - operating microscope
- endoscope assisted
Large tumour with suprssellar extension - raniotomy
Transsphenoidal Surgery omplication
! '
#
*
* #
#
&
(
-
#) '
) #
#
V $"V"
i
i
i# '
i(#
i'
i
(
i|
(
*
"
%
i
%
i
%
i
%
i
º
%
i
%
i
%
!
"
'º
'º
º
,|
#
(
0
"0
%
!
%
i
i
i
i
i
$
%
|
i
i
i
i1
#
i
#%2#
|
/
º
'
0
2
D
ichanges in personality
disinhibition,
inappropriate jocularity,
rage without provocation;
loss of initiative and concern,
apathy,
akinetic mutism,
general retardation
i"frontal release" signs,
i.e. reappearance of primitive reflexes
snout reflex, the grasp reflex, palmar-mental reflex
iunilateral loss of smell (anosmia)
(
iusually involve somatic sensation
iimpairment of tactile sensation
iimpairment of proprioception, i.e. postural sensation and
sensation of passive movement
isensory and visual neglect syndromes, i.e. inability to pay
attention to things in certain parts of the person's sensory or
spatial environment.
This can be as extreme as denial of a limb.
iloss of ability to read, write or calculate
(dyslexia, dysgraphia, dyscalculia)
iloss of ability to find a defined place (geographical agnosia)
iloss of ability to identify objects based on touch
(astereognosia)
(