TUMORS

Question 1

Classification of astrocytoma

Answer 1

diffuse well-differentiated astrocytoma (grade II), anaplastic astrocytoma (grade ill), and glioblastoma (glioblastoma multiforme, or “GBM,” grade N

Question 2

The grade I classification for astrocytomas is reserved for the relatively benign group that includes

Answer 2

pilocytic astrocytomas (well-differentiated tumors mostly of children and young adults); the pleomorphic xanthoastrocytoma (with lipid-filled cells), and the subependymal giant cell astrocytoma (associated with tuberous sclerosis).

Question 3

The ependymomas are subdivided into

Answer 3

cellular, myxopapillary, clear cell, and mixed types

Question 4

a “tumor-like formation that has its basis in maldevelopment”

Answer 4

hamartoma

Question 5

indicate all tumors composed of primitive-looking cells,

Answer 5

blastoma

Question 6

Because of this capacity to transform the cellular genome, the virus product is called an ____; such oncogenes are capable of immortalizing, so to speak, the stimulated cell to form a tumor.

Answer 6

oncogene

Question 7

mutations in the genes that code for i________ are common in gliomas and oligodendrogliomas and their presence relates to less tumor progression

Answer 7

socitrate dehydrogenase(IDH1 and 2)

Question 8

____ have combined deletions in chromosomes 1p and 19q respond well to chemotherapy and this property increases survival

Answer 8

oligodendrogliomas

Question 9

in approximately 50 percent of gliomas there is an overexpression or a mutant form of ______ and of _____, suggesting a role for these in the progression of certain tumor types

Answer 9

epidermal growth factor receptor (EGFR) plateletderived transforming growth factor receptor (PDGFR)

Question 10

alters sensitivity of the glioma to chemotherapy

Answer 10

MGMT, a methyltransferase

Question 11

These high-grade gliomas account for approximately 20 percent of all intracranial tumors and for more than 80 percent of gliomas of the cerebral hemispheres in adults.

Answer 11

G l i o b l a sto m a M u ltifo r m e a n d A n a p l a stic Ast rocyto m a

Question 12

extraneural mets of GBM

Answer 12

bone and lymph nodes

Question 13

gross findings of GBM

Answer 13

variegated appearance, being a mottled gray, red, orange, or brown, depending on the degree of necrosis and presence of hemorrhage, recent or old.

Question 14

histology of GBM

Answer 14

The characteristic histologic findings of glioblastoma are hypercellularity with pleomorphism of cells and nuclear atypia; identifiable astrocytes with fibrils in combination with primitive forms in many cases;

Question 15

This ______ appearance distinguishes glioblastoma from the anaplastic astrocytomas, which show frequent mitoses and atypical cytogenic features but no grossly _____

Answer 15

variegated necrotic or hemorrhagic areas

Question 16

The vasculature and fibroblasts of glioma can undergo a sarcomatous transformation giving the tumor a mixed appearance that is termed ____

Answer 16

gliosarcoma

Question 17

More recently, the progression of astrocytomas and oligodendrogliomas to more malignant forms has been related to ____

Answer 17

the enzyme isocitrate dehydrogenase, encoded by the genes IDH1 and IDH2

Question 18

natural hx of untreated glioblastima

Answer 18

Fewer than 20 percent of patients survive for 1 year after the onset of symptoms and only about 10 percent live beyond 2 years

Question 19

how to give TMZ

Answer 19

The drug is administered daily (75 mg/m2) concurrently with radiotherapy and, after a hiatus of 4 weeks, given for 5 d every 28 d for 6 cycles.

Question 20

high levels of a methyltransferase protein (MGMT) in some glioblastomas lead to_______

Answer 20

resistance to chemotherapy

Question 21

________have been developed in response to the upregulation of EGFR

Answer 21

Tyrosine kinase inhibitors (erlotinib, gefitinib)

Question 22

Almost all glioblastomas recur within _____ of their original site and 10 percent develop additional lesions at distant locations.

Answer 22

2 em

Question 23

Antiangiogenic agents such as________ a VEGF inhibitor, sometimes given in combination with chemotherapy, may delay progression and greatly reduce cerebral edema, therefore diminishing the requirement for corticosteroids, but also do not prolong survival

Answer 23

bevacizumab,

Question 24

_____ is a slowly growing tumor of infiltrative character with a tendency in some cases to form large cavities or pseudocysts

Answer 24

Cerebral astrocytoma

Question 25

In children, astrocytic tumors usually arise in the ________

Answer 25

cerebellum

Question 26

survival rate of cystic astrocytoma of cerebellum

Answer 26

the rate of survival 5 years after successful surgery has been greater than 90 percent

Question 27

variant of high-grade glioma there is a 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

Answer 27

G l i o m atos i s C e re b ri

Question 28

more widespread than the macroscopic picture suggests. If there is a syndrome that can be associated early on with gliomatosis, in our experience it has been a nondescript _______ sometimes mistaken for depression or a subacute dementia, or pseudobulbar palsy may be the first manifestation

Answer 28

frontal lobe behavioral syndrome

Question 29

2 types of ependymoma

Answer 29

one was the ependymoma, and the other, with more malignant and invasive properties, the ependymoblastoma, now recognized as an anaplastic ependymoma

Question 30

There is also a_________ of ependymoma, localized exclusively in the filum terminale of the spinal cord

Answer 30

myxopapillomatous type

Question 31

The most common cerebral site of ependymoma is the ________

Answer 31

fourth ventricle

Question 32

_________are identified by their high mitotic activity and endothelial proliferation, nuclear atypia, and necrosis.

Answer 32

Anaplastic ependymomas

Question 33

The most frequent acquired genetic defects of meningiomas are truncating (inactivating) mutations in the ___________

Answer 33

neurofibromatosis 2 gene (merlin) on chromosome 22q.

Question 34

Meningiomas also elaborate a variety of soluble proteins, some of which _______are angiogenic and probably relate to both the highly vascularized nature of these tumors

Answer 34

(VEGF)

Question 35

Because the clusters of arachnoidal cells penetrate the_______, these are the sites of predilection for the tumor.

Answer 35

dura in largest number in the vicinity of venous sinuses

Question 36

MC meningioma subtype

Answer 36

meningothelial (syncytial)

Question 37

findings of meningioma in angiography

Answer 37

“tumor blush” on angiography