Intracranial Neoplasms and Paraneoplastic Disorders Flashcards
Germ Cell Tumors (6)
Germinoma Embryonal Carcinoma Yolk Sac Tumor (Endodermal Sinus Tumor) Choriocarcinoma Teratoma Mixed Germ Cell Tumors
Most common forms of glioma
astrocytic tumors
Grading represent a spectrum in terms of growth potential
degree of nuclear atypia
cellularity mitoses
vascular proliferation
features of glioblastomas (2)
necrosis and anaplasia of nonneural elements
subdivision of ependymomas (4)
2C
2M
cellular
myxopapillary
clear cell
mixed types
classifications of meningiomas (4)
based on cytoarchitecture and genetic origin
MFAM
meningothelial or syncitial
fibroblastic
angioblastic
malignant
histology: hypercellularity with pleomorphism of cells and nuclear atypia
identifiable astrocytes with fibrils in combination with primitive forms in many cases
tumor giant cells and cells in mitosis
hyperplasia of endothelial cells of small vessels and necrosis hemorrhage and thrombosis of vessels
GBM
variegated appearance distinguish GBM from anaplastic astrocytoma
Genetic Changes in GBM for
- old pts
- young
- amplification of the EGFR gene
2. mutations of the p53 gene
True of False
progression of astrocytomas and oligodendrogliomas to more malignant forms has been related to the enzyme isocitrate degydrogenase , encoded by genes IDH1 and IDH2
True
p645
presence of mutations in the genes that code for IDH 1 and 2 relates to less tumor progression and better outcomes
natural history in GBM
<20% survive one year after onset of symptoms
10% live beyond 2 years
survival with anaplastic astrocytoma in years
3-5 years
cranial irradiationto a total dose of 6000cGy increases survival by ___ months
% months
Stupp trial temozolomide
median survival of 14.6 months with radiation and tmz
12.1 months with radiation
2 year survival 10 to 27%
drug is administered daily 75mg/m2 concurrently with RT and after a hiatus of 4 weeks given 5 d every 28 d for 6 cycles
complications of TMZ
thrombocytopenia and leukopenia in 5 to 10% of patients
rare cases of pneumocystis carinii
response to TMZ if with high levels of methyltransferase protein (MGMT)
resistant to chemo
True or False
Almost all GBM recur within 2cm of their original site and 10 percent develop additional lesions at distant locations
True
Antiangiogenic agents, VEGF inhibitor, sometimes given in combination to chemo may delay progression and greaty reduce cerebral edema
bevacizumab
Median survival for anaplastic astrocytoma
2-5 years or longer
histologic classifications of astrocytoma (4)
- protoplasmic/ fibrillary
- gemistocytic (enlarged cells distended with hyaline and eosinophilic material)
- pilocytic (elongated bipolar cells)
- mixed astrocytoma-oligodendroglioma
T/F
cerebral astrocytoma is a slowly growing tumor of infitrative character with a tendency in some cases to form large cavities or pseudocysts
True
T/F
fine granules of calcium may be deposited in parts of the tumor, but calcium in a slow-growing intracerebral tumor is more characteristic of an oligodendroglioma
True
survival rate after excision of cystic astrocytoma of cerebellum
5 yera survival of greater than 90%
outcome is less assured when the tumor involves the brainstem
prognosis of low grade supratentorial tumors
10 year survival after operation was from 11-40% if with 5,300cGy given postopt
high grade glioma, diffuse infiltration of neoplastic glial cells involving much of one or both cerebral hemispheres with sparing of neuronal elements but without a discrete tumor mass
Gliomatosis cerebri
True or False
Imaging gliomatosis cerebri
contrast enhancement is scant
true
constitute 5 to 7 % of intracranial gliomas
derived from oligodendrocytes or their precursor cells
may occur at any age, most often 30-40
earlier peak at 6-12 yrs
Oligodendroglioma
pink gray color, multilobar relatively avascular and firm (tougher than surrounding brain)
tendency to encapsulate and form calcium and small cysts
Oligodendroglioma
most common site for oligodendroglioma, often deep in white matter with one or more streaks of calcium but little or no surrounding edema
frontal and temporal lobes 40-70 %
intratumoral calcification can be seen in more than half of theses cases
oligodendroglioma
differentials for oligodendroglioma with calcification seen on scan but with the context of seizures
AVM or low grade astrocytoma
oligodendroglioma
loss of certain alleles on chromosome ____ has been predictive of a high degree of responsiveness to _____ chemo regimen
similar loss of chromosome ____ has been associated with longer survival
chrom 1p - responsive to chemo
PCV procarbazine, cyclophosphamide, vincristine)
chrom 19q - with longer survival
type of ependymoma localized exclusively in the filum terminale
myxopapillomatous
Most common glioma of spinal cord
ependymoma
most common cerebral site for ependymoma
4th ventricle
grayish, pink, firm, cauliflower-like growths
in the cerebrum (lateral ventricle) reddish gray, softer and more clearlyd emarcated
originates from the dura mater or arachnoid
meningioma
cells of meningiomas are relatively uniform with round or elongated nuclei, visible cytoplasmic membrane and a characteristic tendency to encircle one another forming
whorls and psammoma bodies
*notable electron microscope characteristic - formation of very complex interdigitations between cells and the presence of desmosomes
most common type of meningioma
meningothelial or syncytial
Most common locations of meningiomas
dural folds
frontoparietal parasagittal convexities, falx, tentorium cerebelli, sphenoid wings, olfactory groove, tuberculum sellae
90% are supratentorial
Grading of astrocytoma tumors
Grade I: pilocytic astrocytoma, pleomorphic xanthoastrocytomas, subependymal giant cell astrocytomas
Well-differentiated astrocytoma Grade II
Anaplastic Astrocytoma Grade III
GBM Grade IV
astrocytoma associated with Tuberous sclrerosis
subependymal giant cell astrocytomas
choroid plexus tumors divided into two classes
papillomas and carcinomas
50% of astrocytomas have deletions within this gene
tumor suppressor gene p53
After development of astrocytoma, progression to a more malignant grade may be triggered by defects in the: (3)
p16-retinoblastoma gene signaling pathway
Loss of chromosome 10 (90% of high-grade glioma)
over expression of Epidermal Growth Factor gene
T/F
Oligodendrogliomas that have combined deletions in
chromosomes 1p and 19q respond well to chemotherapy
and this property increases survival.
True
p645
Which of the ffg means good survival/less tumor progression
a. mutations in the genes that code for IDH 1 and 2 in gliomas and oligodendrogliomas
b. combined deletions in chrom 1p and 19q in oligodendrogliomas
c. amplifications of MYCN gene
d. A and B
e. B and C
D. A and B
amplification of MYCN oncogene - aggressive course, poor outcome in neuroblastoma and medulloblastoma
p645
VEGF is found in extremely high concentrations in
Meningiomas
Imaging findings of vasogenic vs cytotoxic edema
both cytotoxic and vasogenic
decreased attenuation on CT
hyperintensity on T2 MRI
cytotoxic : reduced diffusivity on DWI (increased anisotropy)
vasogenic: elevated diffusivity (reduced anisotropy)
dosing of Mannitol 25% solution
- 5 to 1.0g/kg over a period of 2 to 10 mins
* hyeprtonic saline are equally effective, 3,7, 23 %
characteristics of headache in brain tumor
slight, dull, episodic
dull or sharp, intermittent
nocturnal occurrence, presence on first awakening, deep, nonpulsatile quality
BUT these are not specific
Convulsions have of been observed in ___ to ____% of all patients with cerebral tumors
20-50%
T/F
A first seizure during adulthood is always suggestive of brain tumor and, in the authors’ experience, has been the most common initial manifestation of primary and metastatic neoplasm.
True
p649
T/F
In high grade gliomas
Malignant cells carried in the CSF may rarely form distant foci on spinal roots or cause a widespread meningeal gliomatosis.
True
p.650
T/F
In high grade gliomas
Extraneural metastases involving the bone and lymph nodes are common.
False
very rare
p650
How many percent of GBM
occupy more than one lobe of a hemisphere?
show multicentric foci of growth, simulating metastases
occupy more than one lobe of a hemisphere - 50%
show multicentric foci of growth, simulating metastases - 3-6%
Management in GBM
feasible resection, combined with chemo and RT
chemo - carmustine or lomustine increase survival slightly
cisplatin, carboplatin
-GMT group: clear but small benefit of chemo
tyrosine kinase inhibitors for GBM
erlotinib, gefitinib
Survival in GBM with
aggressive surgical removal and radiotherapy
no treatment
aggressive surgical removal and radiotherapy - 12 months
no treatment - 7-9 months