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Unusual glioma
1. Unusual Gliomas
Dr vardan kulshreshtha
M.cH Resident â final year
Deptt. Of Neurosurgery
RNT Medical College , Udaipur, INDIA
10/20/2016 1unusual giomas
2. ⢠These tumor deserve special consideration
⢠These are rare and there behaviour and
natural history is still largely less known
⢠Many of these have indolent growth and
commonly reach to attention due to epilepsy
⢠Routine use of IHC and genetic assay have led
to some understanding.
10/20/2016 2unusual giomas
3. Subependymal Giant Cell Astrocytoma
⢠WHO grade 1
⢠Generally occur in
patients with tuberous
sclerosis
⢠Typical location is
intraventricular
⢠Present with features of
raised ICP and
obstructive HCP
⢠TUBEROUS Sclerosis is
an autosomal dominant
disease
⢠Mental retardation,
seizures, adenomas
sebaceum
⢠Altered skin
pigmentations, retinal
tumors, subungual
fibromas, tumors of
pancreas and spleen
10/20/2016 3unusual giomas
4. Pathology
⢠Rarely undiagnosed
⢠Found typically in
intraventricular locations
⢠Typical appearance of giant
cells mixed with cells of
astrocyte lineage
⢠Well demarcated tumors
with modest infilteartion
into surrounding white
matter.
⢠Giant cells with abundant
eosinophilic cytoplasm and
spindle shaped nuclei
⢠Mitoses, nuclear atypia,
necrosis, endothelial
proliferation with
aggressive behaviour
⢠Anaplastic transformation
is rare
⢠IHC marker show positivity
for GFAP. AND S-100
10/20/2016 4unusual giomas
6. MRI
⢠Isointense to
hypointense on T1 mass
in the periventricular
location
⢠Hyperintense signal on
T2 WI.
⢠Strongly enhanced
signals on post contrast
10/20/2016 6unusual giomas
7. Management
⢠Do not require intervention
unless bolck the foramen of
monroe
⢠Primary treatment is
surgical/ total resection
⢠Subtotal resection may be
adequate in case total
resection is hazardous
⢠Tumor rarely becomes
aggressive
⢠Tarnscortical/ transcallosal
approach
⢠If surgery not possible or
denied by pt. then
management of HCP by
shunt
⢠Role of chemotherapy has
shown promises
10/20/2016 7unusual giomas
8. Everolimus for Subependymal Giant-Cell Astrocytomas in Tuberous Sclerosis
Darcy A. Krueger, M.D., Ph.D., Marguerite M. Care, M.D., Katherine Holland, M.D., Ph.D., Karen
Agricola, F.N.P., Cynthia Tudor, P.N.P., Prajakta Mangeshkar, M.S., Kimberly A. Wilson, M.S.,
Anna Byars, Ph.D., Tarek Sahmoud, M.D., Ph.D., and David Neal Franz, M.D.
N Engl J Med 2010; 363:1801-181
⢠Background
⢠Neurosurgical resection is the standard treatment for subependymal giant-cell astrocytomas in patients with the tuberous
sclerosis complex. An alternative may be the use of everolimus, which inhibits the mammalian target of rapamycin, a
protein regulated by gene products involved in the tuberous sclerosis complex
⢠Methods
⢠Patients 3 years of age or older with serial growth of subependymal giant-cell astrocytomas were eligible for this open-label
study. The primary efficacy end point was the change in volume of subependymal giant-cell astrocytomas between baseline
and 6 months. We gave everolimus orally, at a dose of 3.0 mg per square meter of body-surface area, to
achieve a trough concentration of 5 to 15 ng per milliliter.
⢠Results
⢠We enrolled 28 patients. Everolimus therapy was associated with a clinically meaningful reduction in volume of the
primary subependymal giant-cell astrocytoma, as assessed on independent central review (P<0.001 for baseline vs. 6
months), with a reduction of at least 30% in 21 patients (75%) and at least 50% in 9 patients (32%). Marked reductions
were seen within 3 months and were sustained. There were no new lesions, worsening hydrocephalus, evidence of
increased intracranial pressure, or necessity for surgical resection or other therapy for subependymal giant-cell
astrocytoma. Of the 16 patients for whom 24-hour video electroencephalography data were available, seizure frequency
for the 6-month study period (vs. the previous 6-month period) decreased in 9, did not change in 6, and increased in 1
(median change, â1 seizure; P=0.02). The mean (ÂąSD) score on the validated Quality-of-Life in Childhood Epilepsy
questionnaire (on which scores can range from 0 to 100, with higher scores indicating a better quality of life) was
improved at 3 months (63.4Âą12.4) and 6 months (62.1Âą14.2) over the baseline score (57.8Âą14.0). Single cases of grade 3
treatment-related sinusitis, pneumonia, viral bronchitis, tooth infection, stomatitis, and leukopenia were reported..
⢠Conclusions
⢠Everolimus therapy was associated with marked reduction in the volume of subependymal giant-cell
astrocytomas and seizure frequency and may be a potential alternative to neurosurgical resection in some
cases, though long-term studies are needed.
10/20/2016 8unusual giomas
9. Angiocentric glioma
⢠Described by WHO in
2005
⢠Initially included with
astroblastoma and
choroid glioma
⢠Mean age at diagnosis is
17 years
⢠Pts present with long
history of seizures
⢠Microscopically show the
monomorphous , bipolar
cells associated with
normal vessels of the
cortex and white matter
⢠Tumor cells are spindle
shaped
⢠Mitoses are rare
⢠Positive for S-100, GFAP,
vimentin
10/20/2016 9unusual giomas
10. Imaging
⢠CT images show it as a
hypodense lesion at the
cortex
⢠MR shows a mass with
stalk towards the
ventricular surfaceâ
pathognomic
⢠Hypointense to iso on
T1WI
⢠Hyperintense on T2WI
⢠Post contrast
enhancement is sparse
10/20/2016 10unusual giomas
12. Management and outcome
⢠Follow an indolent course
⢠Rare progression
⢠Surgical cure obtained by total resection
⢠Aggressive forms are exceedingly rare
10/20/2016 12unusual giomas
13. ⢠Perm J. 2013 Winter;17(1):e100-2. doi: 10.7812/TPP/12-060.
⢠The treatment of angiocentric glioma: case report and literature review.
⢠Alexandru D1, Haghighi B, Muhonen MG.
⢠Abstract
⢠Angiocentric glioma is a recently described tumor recognized since 2007
by the World Health Organization Classification of Tumours of the Central
Nervous System. We present the only case of angiocentric glioma at our
institution in the last 15 years and review the literature in an attempt to
establish prognostic parameters. Our search revealed only 27 cases of
angiocentric glioma in the literature. The most common presenting
symptom of angiocentric glioma was seizures. Gross total resection of
the lesion was curative, without need for radiation or chemotherapy.
10/20/2016 13unusual giomas
14. Astroblastoma
⢠WHO grade 1
⢠Initially described by Bailey
and Bucy in 1930
⢠Has been a matter of debate
for similar features as
anaplastic astrocytoma,
glioblastoma and
gemistocytic astrocytomas
⢠Commonly found in 1st
three decades of life
⢠Female
preponderance(11:1)
⢠Affects the hemispheres
involving cortex ,
subcortical and
periventricular regions
⢠No infratentorail tumor
have been reported
⢠Pts present with features of
raised ICP, cortical deficits,
seizures, personality
changes
10/20/2016 14unusual giomas
15. ⢠Greatly variable natural
history
⢠Have a slow and
indolent course
⢠Rarely show an
aggressive outcome
⢠Almost always defined
by their gross
appearance
⢠Cut surface reveal
homogenous soft pink
grey substances
⢠Cystic areas are
frequently encountered
⢠The clusters of cells
form pseudorossette
10/20/2016 15unusual giomas
16. ⢠Richly supplied by
blood vessels
⢠Nuclei of tumor cells
are away from blood
vessels and send fine
processe to the vessel
wall
⢠High number of mitotic
figures can be found
⢠Cellular atypia is
frequently noted
⢠On IHC tumor cells
stain positive for GFAP
⢠Positivity for vimentin,
NSE, S-1OO, EMA
⢠These are positive for
anti leu 7 antibodies
specific for cells of
epithelial origin
⢠Central necrosis can be
found
10/20/2016 16unusual giomas
17. Imaging
⢠On CT the appearnaces
varies from poorly
defined hypodense
tumor to well
demarcated and highly
enhancing masses
10/20/2016 17unusual giomas
18. ⢠On MR image tumors
are hypointense to
white matter on T1
⢠Hyperintense on T2
⢠Hyperintense on FLAIR
⢠Enhancement is seen
around the cystic centre
10/20/2016 18unusual giomas
19. Management And Prognosis
⢠Surgical resection is main treatment
⢠Gross total resection can be easily achieved as
lesions are mostly in the cortical areas
⢠Therapeutic value is of radiation and
chemotherapy is uncertain as these are rare
tumors
⢠Prediction of outcome is difficult as these remain
indolent for a long time and may rapidly
transform to glioblastoma resulting in fatal
outcomes.
10/20/2016 19unusual giomas
20. Pilomyxoid astrocytoma
⢠Recently described tumors
⢠Similarities with pilocytic
astrocytomas
⢠Until recently these were
grouped with PA
⢠These involve entire
neuraxis
⢠Mean age at preentation is
18 months
⢠Most are found in
hypothalamic â chiasmic
region
⢠Pts present with features of
raised ICP
⢠RAISED HEAD
CIRCUMFERENCE may be
only subtle sign in infants
⢠Histologically consists of
myxoid matrix with cells in
loose fibrillary and myxoid
background
⢠Tumor cells form
perivascular rosettes
10/20/2016 20unusual giomas
21. ⢠Preferred sites include:
⢠(1) optic nerve (âoptic
nerve gliomaâ)
⢠(2) optic
chiasm/hypothalamus
⢠(3) thalamus and basal
ganglia
⢠(4) cerebral hemisphere
⢠(5) cerebellum (âcerebellar
astrocytomaâ) and
⢠(6) brainstem (dorsal
exophytic brainstem
glioma)
⢠Genetically chracterized by
the presence of NF1
mutations, BRAF
duplications and the
absence of IDH1 mutation
10/20/2016 21unusual giomas
22. Imaging
⢠The huge chiasmatic-
hypothalamic tumor reveals
low signal intensity on a
T1-weighted image (A)
⢠Bright signal intensity on a
T2-weighted image (B).
⢠Gadolinium-enhanced
brain MR images show
heterogeneously strong
enhancement (C and D)
⢠Leptomeningeal
disseminations at the
cervical spine levels
10/20/2016 22unusual giomas
23. ⢠NO standard care
protocols have been
defined
⢠As these are newly
defined and rare
tumors
⢠Surgical resection is
favoured if location
favours
⢠Mean progression free
survival is 25 months
⢠Mean overall survival is
60 months
⢠Reduced survival is
because of inability to
achieve gross resection
10/20/2016 23unusual giomas
24. ⢠The usual indications for
adjuvant treatment
(chemotherapy or
radiation therapy)
include tumor recurrence
after initial complete
resection or symptomatic
tumors
⢠In addition, adjuvant
treatment is generally
indicated for tumors with
growth on follow-up
imaging, even in the
absence of symptoms.
⢠The role of chemotherapy
is in evolution
⢠Chemotherapy may be
implemented for the treatment of
inoperable or partially resected
gliomas
⢠The decision to use postoperative
adjuvant therapy also depends on
a lesion's perceived malignant
tendencies, as the potential
benefits of radiation therapy and
chemotherapy must be weighed
against their morbidity
⢠The decision to use postoperative
adjuvant therapy also depends on
a lesion's perceived malignant
tendencies, as the potential
benefits of radiation therapy and
chemotherapy must be weighed
against their morbidity
10/20/2016 24unusual giomas
25. Pleomorphic xanthoastrocytoma
⢠First described by Keps
and colleuges in 1979
⢠Represents <1% of all
astrocytomas
⢠May be found along
entire neuraxis, mostly
supratentorial( temporal
lobe)
⢠Cerebellum and soinal
cord are the other sites
⢠Pts present in 2nd and 3rd
decades
⢠Median age at 14 years
⢠Both sexes are affected
equally
⢠70-80% pts present with
seizures and headache,
focal neuro deficits
⢠Rarely tumor may
hemmorrhage
10/20/2016 25unusual giomas
26. ⢠Supratentorail are most
often cortical and meninges
based
⢠Chronic seizures
⢠Grossely these are firm
tumors, variable in color
relatively avascular
⢠Typically invade the pia
arachnoid space
⢠13% pts have involvement
of all the three meninges
⢠Cyst fluid is xanthochromic
⢠Very specific microscpoic
criteria for diagnosis are:
1. Pleomorphic and
xanthomatous astrocytes
2. Perivascular lymphocytes
3. Non infilterating histology
4. Reticulin staining
⢠Pleomorphism can be as
vast as glioblastoma or
sarcomatous tumors
10/20/2016 26unusual giomas
27. ⢠IHC Staining reveals
spindle cells and round
pleomorphic cells
positive for S-100 and
GFAP
⢠Staining for
synaptophysin and NSE
may be also present
⢠Some workers have
reported them to be a
subtype of ganglioglioma
⢠Association between the
Neurofibromatosis ,
Sturge Weber Syndrome
⢠Loss of chr. 9 and gain of
chromosome 7 have been
found
10/20/2016 27unusual giomas
28. Imaging
⢠CT
⢠PXAs are typically hypo
or isodense and may be
well or poorly
demarcated, usually with
no or little surrounding
oedema.
⢠Calcification is rare
⢠Due to its superficial
location it may cause
scalloping of the
overlying bone
10/20/2016 28unusual giomas
29. ⢠MRI
⢠T1
â solid component iso to
hypointense c.f. grey matter
â cystic component low signal
â leptomeningeal involvement
seen in over 70% of cases
⢠T1 C+ (Gd)
â solid component usually
enhances vividly
⢠T2
â solid component iso to
hyperintense c.f. grey matter
â cystic component high signal
â on T2 FLAIR sequence, cystic
areas show hyperintensity
relative to CSF due to higher
protein contents
â little surrounding vasogenic
oedema
10/20/2016 29unusual giomas
30. Management & Outcome
⢠Quite rare tumors
⢠Surgical excision appears to
be best favoured modality
⢠Actuarial survival trates are
91% at 5 years and 82% at
10 years
⢠Median survival 18 years
⢠Total resection should be
attempted in every case as
far as possible
⢠Resection of cyst lining is
not indicated
⢠Data regarding the use of
adjuvant therapy at this
time are limited to few case
reports only
⢠Pts who had incomplete
resection, high mitotic
index , necrosis should be
reserved for strict vigilance
and CEMRI on follow ups
10/20/2016 30unusual giomas
31. ⢠At progression repeat
resection be attempted
⢠Proression should be
taken as aggressive
tumor type
⢠Malignant
transformation may
necessitate adjuvant
therapy
10/20/2016 31unusual giomas
32. ⢠Br J Neurosurg. 2013 Dec;27(6):759-64. doi: 10.3109/02688697.2013.776666. Epub 2013 Mar 20.
⢠Pleomorphic xanthoastrocytoma: long-term results of surgical treatment and analysis of prognostic
factors.
⢠Gallo P1, Cecchi PC, Locatelli F, Rizzo P, Ghimenton C, Gerosa M, Pinna G.
⢠BACKGROUND:
⢠Pleomorphic Xanthoastrocytoma (PXA) is a rare brain tumour, most commonly affecting children and
young adults. To date, only few data regarding the long-term follow-up of these patients after surgery are
available. The aim of this study is to describe our single-institution experience in the surgical
management of this particular glioma over a period of over 18 years.
⢠METHODS:
⢠We performed a retrospective review of all cases of PXA (40 patients) operated upon at the
Department of Neurosurgery of Verona, Italy, between 1990 and 2008. The impact of clinical, radiological,
surgical and histological factors on overall survival (OS) and progression-free survival (PFS) was analysed by
means of univariate and multivariate models.
⢠FINDINGS:
⢠We achieved a gross total resection (GTR) in 65% of patients. Histological diagnosis was of grade II in 80%;
anaplastic features were present in the remaining 20%. Adjuvant treatment, radiotherapy or chemo-
radiotherapy, was administered in 40% of the cases. Median follow-up was 74 months. OS at 5- and
10 years was 76.32% and 68.24%, respectively. PFS at 5- and 10 years was 71% and 58%,
respectively. In the multivariate model, histological grade, extent of resection and age at diagnosis (⤠30
years vs > 30 years) were the only independent prognostic factors for both OS and PFS.
⢠CONCLUSIONS:
⢠Our retrospective long-term study confirms the relatively favourable prognosis associated
with PXA. Young patients with a low-grade tumour (WHO grade II) who underwent GTR carry
the longest OS and PFS.
10/20/2016 32unusual giomas
33. ⢠Brain Tumor Pathol. 2006 Apr;23(1):55-63.
⢠A case of anaplastic pleomorphic xanthoastrocytoma presenting with tumor bleeding and
cerebrospinal fluid dissemination.
⢠Asano K1, Miyamoto S, Kubo O, Kikkukawa T, Yagihashi A, Ohkuma H.
⢠Pleomorphic xanthoastrocytoma (PXA) has been considered an astrocytic tumor
with a relatively favorable prognosis. However, PXA cases having several recurrent
patterns with poor prognosis have been reported in recent years, and a new concept of
anaplastic PXA has been proposed. The present case was a 59-year-old woman who
presented with tumor bleeding onset and cerebrospinal fluid dissemination. The patient had
sudden-onset right hemiparesis, aphasia, and consciousness disturbance and was admitted
to a local area hospital. After emergency surgery had removed the hematoma, postoperative
contrast-enhanced CT scan revealed a left temporal tumor. A second surgery was therefore
performed for initial tumor removal 2 months later. Histopathological findings showed that
the tumor was typical PXA with strong pleomorphism and xanthomatous changes and
contained an ependymoma-like component in the center area. However, endothelial
proliferation and mitosis were more remarkable compared to ordinary PXA. The MIB-1
labeling index was 9.8% high. From these findings, the histopathological diagnosis was
anaplastic PXA. The patient underwent surgery to remove recurrent tumors 5 and 16 months
later. The patient died 36 months after the first onset, and CT revealed glioblastoma-like
findings and cerebrospinal fluid dissemination. This case report is the first case in which
PXA presented with tumor bleeding onset. Histopathological findings suggested
anaplastic PXA from the first surgical specimens, and PXA recurred many times. We
thus believe that the patient displayed primary anaplastic PXA rather than
secondary anaplastic PXA that results in malignant transformation.10/20/2016 33unusual giomas
34. Ganglioglioma
⢠WHO grade 1
⢠Initially described by
Perkins in 1926
⢠Benign , well
differentiated
neuroepithelial tumors
⢠Most commonly found in
temporal lobe though can
occur anywhere in CNS
⢠Commonly found in
young patients with
epilepsy
⢠Most commonly noted
tumor in temporal lobe
epilepsy patients
⢠Have been described in
brain steem , spinal cord
and cerebellum
⢠Account for 1% of
primary brain tumors
10/20/2016 34unusual giomas
35. ⢠Male dominence
⢠Most common features
are seizures, headache,
dizziness, ataxia,
progressive weakness
⢠Malignant
transformation is rare
⢠Histology shows
immature mixture of
abnormal neural and
glial elements
⢠Macroscopically appear
solid or cystic
⢠Cystic are well
delineatedfrom
surrounding brain
⢠Calcification is common
10/20/2016 35unusual giomas
36. ⢠IHC staing shows reactivity for GFAP, S-100 and
vimentin
⢠Neurons show synaptophysin, class III beta
tubulin, NFP , chromogranin reactivity
10/20/2016 36unusual giomas
37. Imaging
⢠Highly variable imaging
characters
⢠No special typical
imaging criteria
⢠Enhancement patterns
are highly variable
⢠Iso to hypointense on
T1WI
⢠Heterogenous and
hyperintense on T2WI
⢠Can contain solid, cytic
calcified components
10/20/2016 37unusual giomas
38. ⢠FDG PET scan shows
hypometabolism or
eumetabolism in these
patients
10/20/2016 38unusual giomas
39. Management And Outcome
⢠Gold standard is gross
total resection
⢠Seizure control after
surgery is as high as
80%
⢠Survival rates are high
⢠Event free survival of
low grade and high
grade gangiogioma are
57% vs 15%.
⢠The optimal surgical
resection depends upon
the eloquent areas
involved in the vicinity
of the tumor
⢠The use of
chemotherapy is not
well established
⢠Typically they should
be allowed for chemo
and radiotherapy
10/20/2016 39unusual giomas
40. ⢠The use of adjuvant therapy is warranted in those
cases which harbour residual lesions or features of
anaplasia
⢠Potential harm to nearby brain areas preclude the
judicious use of radiation
10/20/2016 40unusual giomas
41. ⢠Brain Tumor Res Treat. 2014 Oct; 2(2): 49â55.
⢠Treatment and Outcomes for Gangliogliomas: A Single-Center Review of 16 Patients
⢠Jye Young Song, Jeong Hoon Kim, Young Hyun Cho, Chang Jin Kim, and Eun Jung Lee
⢠Ganglioglioma is a rare and slowly growing benign tumor. We investigated the outcomes of patients who
underwent different combination treatments.
⢠Methods
⢠Between 1998 and 2012, 16 patients, including 11 men and 5 women, with a median age of 12.5 years (range, 2.5-
65 years) were treated for intracranial gangliogliomas at our institution. The median follow-up period was 5.7
years (range, 48 days-15.6 years). Fifteen cases were included in the outcome assessment because one patient
was lost to follow-up. Complete resection was achieved in 8 (53%) patients. Six (40%) patients underwent
incomplete resection with or without adjuvant radiotherapy, and one patient with a brainstem tumor underwent
only stereotactic biopsy.
⢠Results
⢠Gangliogliomas predominantly affected young (87.5%), male patients and most frequently presented with seizures
(64%). Of eight patients who underwent complete resection, seven did not show recurrence, whereas only three
of six with incomplete resection showed no recurrence. Four patients with recurrence received salvage treatments
(two repeat surgeries and two radiosurgeries). A tumor control rate of 93% (14/15) was achieved at the last
follow-up. No recurrence or malignant changes were observed after a median follow-up of 12 and 4.5 years in four
patients who received gamma knife (GK) radiosurgery as adjuvant and salvage treatment.
⢠Conclusion
⢠Complete resection produced the best outcomes and incomplete resection followed by adjuvant or
salvage treatments showed favorable outcomes. In patients who are not eligible for complete
resection because of tumor location or potential neurologic deficits following surgery, GK
radiosurgery should be considered for the treatment of residual or recurrent tumors.
10/20/2016 41unusual giomas
42. Papillary tumours of pineal
⢠WHO grade II AND III
⢠Relatively new edition to WHO
⢠They are equal in both sexes
⢠Reach medical attention due to headache, mental
status changes and altered vision
⢠Contain eosinophilic cells with distinctive
borders with large pleomorphic nuclei
⢠Positive for cytokeratin, synaptophysin, stain
variably for GFAP, vimentin, S-100
10/20/2016 unusual giomas 42
43. Imaging
⢠Generally found to be diffusely enhancing
lesion of pineal region
⢠Post 3rd ventricle with HCP
⢠Appear well demarcated from thalamus and
cerebellum
10/20/2016 unusual giomas 43
44. Mangement
⢠Standard treatment is excision
⢠Grow slowly and donot transform to
malignant tumors
⢠Local recurrence is however possible
⢠Radiotherapy appears to provide good control
in local recurrence
10/20/2016 unusual giomas 44
45. Choroid Glioma Of Third Ventricle
⢠WHO Grade II
⢠Ist recognised by WHO in
1998
⢠Arise in the anterior third
ventricle
⢠Commonly come to
attention after HCP
⢠Appera to contain in 3rd
ventricle and thought to
arise from ventricular
surface
⢠Composed of GFAP and
vimentin positive cells in
mucinous matrix
⢠Nuclei are uniform in
size and shape
⢠Indolent growth
⢠May show EGF receptor
positivity
⢠Some suggets these tumor
arise from the lmina
terminalis and
ventricular tanycytes
10/20/2016 45unusual giomas
46. Imaging
⢠Commonly fill 3rd
ventricle
⢠Diffusely enhance with
contrast
⢠Appear to infiltrate the
thalamus
⢠Large tumors may be
difficult to differentiate
from suprasellar masses
10/20/2016 46unusual giomas
48. ⢠Surgical management is limited by location
⢠Access through the dialted ventricle and lamina
terminalis may be challanging
⢠Subtotal resection is thus common
⢠Role of adjuvant radiotherapy is unknown
10/20/2016 48unusual giomas
49. ⢠Acta Neurochir (Wien). 2008 Nov;150(11):1183-91. doi: 10.1007/s00701-008-0014-6. Epub
2008 Oct 21.
⢠Chordoid glioma of the third ventricle.
⢠Vanhauwaert DJ1, Clement F, Van Dorpe J, Deruytter MJKGROUND:
⢠Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle
with both glial and chordoid features. It was first described by Brat in 1998. Since there is no detailed
information available on the outcome after surgery and adjuvant treatment, we reviewed the literature.
⢠METHODS:
⢠A literature search through PUBMED revealed 50 cases of chordoid glioma. Most reports
were found in pathology journals. Information on the postoperative course was sometimes
very limited. We reviewed the available literature and studied in detail the presenting
symptoms, mortality and postoperative complications in relation to the extent of resective
surgery, as well as the importance of adjuvant treatment.
⢠CONCLUSIONS:
⢠Mortality in the immediate postoperative period is 32% and is higher after gross total
resection as compared to subtotal resection. Non-fatal postoperative complications are
hypothalamic disorders and mental alterations. Gross total resection is the treatment of
choice since no recurrence has been reported after macroscopically complete resection,
but this is often difficult because of the location and adherence to the hypothalamus. The
role of postoperative radiotherapy is uncertain. There is some indication that radiosurgery
with or without conventional irradiation is superior to conventional radiation alone. Planned
subtotal resection followed by stereotactic radiosurgery can be a safe and effective
alternative in a patient in whom gross total resection is considered to be too risky.
There is no report on the use of chemotherapy in the treatment of chordoid gliomas. More
information about the optimal treatment strategy is needed, and more reports are also needed.10/20/2016 49unusual giomas
50. ⢠Neurologist. 2011 Jan;17(1):52
⢠Chordoid glioma: a case report and literature review.
⢠Liu WP1, Cheng JX, Yi XC, Zhen HN, Fei Z, Li Q, Zhang X.
⢠chordoid glioma is a rare tumor (World Health Organization grade II) with both
glial and chordoid features, often located in the suprasellar region and anterior
third ventricle. It was first described by Brat in 1998. Because there is no detailed
information available from the clinical perspective, we reviewed the literature.
⢠METHODS:
⢠a literature search through PUBMED and CNKI revealed 64 cases of
chordoid glioma. Information on the clinical course was very limited. We
reviewed the literature and studied the pathologic and imaging features,
postoperative mortality and morbidity in relation to surgical extension and
approaches, and the importance of adjuvant treatment.
⢠CONCLUSIONS:
⢠mortality in the immediate postoperative period is 28%, and postoperative
morbidity is 60%, which are statistically higher after gross total resection as
compared with subtotal resection. Translamina terminalis approach is considered
to be the best approach. The current study cannot document that patients have
longer survival and higher quality of life after gross total resection than subtotal
resection. The role of postoperative radiotherapy is uncertain and there is
no report on the use of chemotherapy. More information about the optimal
treatment strategy is needed.
10/20/2016 50unusual giomas
51. Conclusion
⢠They are identified by their unique morphological
features
⢠Many of these have indolent course and identified by
epilepsy
⢠Potentail diagnosis should be considered in young
patients with seizures and cortically based lesions.
⢠Correct diagnosis requires experienced
neuropathologisrt
⢠As more experienced is gained more knowledge
towards better understanding shall provide better
outcomes in these patients
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