TUMORS2

Question 1

Almost ______ of all leukemic patients have evidence of diffuse infiltration of the leptomeninges and cranial and spinal nerve roots at autopsy

Answer 1

one-third

Question 2

The incidence is greater in acute than in chronic leukemia and greater in ______ than in myelocytic leukemia

Answer 2

lymphocytic

Question 3

The highest incidence of involvement of CNS is in children with _______ who relapse after treatment with combination chemotherapy (60 to 70 percent at time of death)

Answer 3

acute lymphocytic (lymphoblastic) leukemia

Question 4

The studies of Price and Johnson demonstrated that CNS leukemia is primarily a ________. The earliest evidence of leukemia is detected in the walls of pial veins, with or without cells lying freely in the CSF

Answer 4

pial disease

Question 5

_______ and __________ given intrathecally or intravenously; has been effective in the prevention and treatment of meningeal involvement in childhood leukemia

Answer 5

Cranial radiation, combined with methotrexate

Question 6

Cx of chemoRT after tx of meningeal involvement in childhood leukemia

Answer 6

necrotizing leukoencephalopathy within several days to months after the last administration of methotrexate and several months after completion of radiotherapy

Question 7

MRI findings of necrotizing leukoenceph vs radiation necrosis

Answer 7

On MRI these lesions appear T2 hyperintense, but compared with pure radiation necrosis (see further on), they have poorly demarcated borders and like on CT, do not enhance

Question 8

__________ o f the spinal cord o r cauda equina is the most common neurologic complication of all types of lymphoma, the result of extension from vertebrae or paravertebral lymph nodes

Answer 8

Extradural compression

Question 9

In the rare cases of meningeal involvement with _______, there may be an eosinophilic pleocytosis

Answer 9

Hodgkin lymphoma

Question 10

These are malignant tumors composed of cells derived from connective tissue elements (fibroblasts, rhabdomyocytes, lipocytes, osteoblasts, smooth muscle cells

Answer 10

S a rc o m a s of t h e B ra i n

Question 11

_______ have occurred after radiation of pituitary adenomas and osteogenic sarcoma after other types of radiation, all localized to bone or meninges.

Answer 11

Fibrosarcomas

Question 12

_________is an invasive and rapidly growing tumor, mainly of childhood, that arises in the posterior part of the cerebellar vermis and neuroepithelial roof of the fourth ventricle in children

Answer 12

Medulloblastoma

Question 13

Origin of Medulloblastoma

Answer 13

The current view of the tumor is that it originates from pluripotential stem cells (that can differentiate into neuronal or glial elements) that have been prevented from maturing to their normal growth-arrested state

Question 14

Certain molecular genetic similarities relate the medulloblastoma to _________, ____________ and, rarely, to autosomal dominant diseases such as ________

Answer 14

retinoblastomas and pineal cell tumors, nevoid basal cell carcinoma.

Question 15

Chromosomal studies of medulloblastomas reveal a deletion on the distal part of chromosome _______ distal to the p53 region.

Answer 15

1 7

Question 16

It is also notable that medulloblastomas are encountered in ______, caused by mutations in the gene encoding “patched,” the receptor for sonic hedgehog ligand, and in ________, as a consequence of mutations in DNA repair genes

Answer 16

Gorlin syndrome Turcot syndrome

Question 17

In Medulloblastoma: Gene expression profiling has given preliminary evidence that amplification or overexpression of the transcription factor ________ is associated with a poorer prognosis (as it is in neuroblastoma)

Answer 17

MYCN (N-MYC)

Question 18

In Medulloblastoma, The majority of the patients are children ______years of age, and males outnumber females_______ in most reported series

Answer 18

4 to 8 3:2 or 3:1

Question 19

In Medulloblastoma,

Answer 19

The radiologic appearance is also distinctive: high signal intensity on both Tl- and T2-weighted MRis, heterogeneous enhancement, and, of course, the typical location adjacent to and extending into the fourth ventricle

Question 20

________ of the tumor is recommended. The addition of________ and ________ of the entire neuraxis improves the rate and length of disease-free survival even for those children with the most extensive tumors at the time of diagnosis

Answer 20

Maximal resection chemotherapy and radiotherapy

Question 21

In Medulloblastoma, The combination of surgery, radiation of the entire neuraxis, and chemotherapy permits a 5-year survival in more than______ percent of cases

Answer 21

80

Question 22

In Medulloblastoma, The presence of__________ (i.e., connective tissue formations) is associated with a better prognosis independent of the type or treatment

Answer 22

desmoplastic features

Question 23

In Medulloblastoma, Any of the features of _______, ________, _______ greatly reduce the period of survival.

Answer 23

brainstem invasion, spinal subarachnoid metastases, incomplete removal, and very early age of onset (younger than 3 years)

Question 24

This, the most common solid tumor of childhood, is a different entity from medulloblastoma but of nearly identical histologic appearance, arising in the adrenal medulla and sometimes metastasizing widely.

Answer 24

Neuroblastoma

Question 25

Paraneoplastic complication of NB

Answer 25

polymyoclonus with opsoclonus and ataxia

Question 26

T or F A rare form of neuroblastic medulloblastoma in adults tends to be more benign

Answer 26

T

Question 27

Poor prognosticating factors in NB

Answer 27

1. loss of heterozygosity of certain sites on chromosomes 1 and 11 2. MYCN amplification or overexpression 3. polymorphisms in chromosome 6p`

Question 28

Treatment of NB low intermediate high

Answer 28

Treatment is predicated on clinical staging, with the lowest risk category allowing observation because some lesions regress spontaneously. Those patients who are at intermediate risk are treated with chemotherapy and high risk children have surgical resection and receive intensive chemotherapy, radiation, and in selected cases, hematopoietic stem cell transplantation

Question 29

SR of patients with NB

Answer 29

survival rates exceeding 90 percent, but the highest risk group has a 30 percent survival rate

Question 30

This proves to be one of the most frequent extracranial malignant tumors of infancy and childhood

Answer 30

RB

Question 31

It is a small cell tumor with neurofibrils and, like neuroblastoma, has a tendency to form rosettes.

Answer 31

RB

Question 32

Whereas the supratentorial ependymoma occurs at all ages, _____ ependymomas appear mostly in childhood.

Answer 32

fourth ventricular

Question 33

The most anaplastic form of ependymoma is the \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_, a highly aggressive tumor that falls within the spectrum of primitive neuroectodermal tumors

Answer 33

ependymoblastoma

Question 34

Locations of choroid plexus papilloma

Answer 34

They arise mainly in the lateral and fourth ventricles, occasionally in the third.

Question 35

Molecules associated with tumor induction in choroid papilloma

Answer 35

T (tumor) antigen of the SV40 virus is possibly involved in tumor induction

Question 36

MRI appearance of hemangioblastoma

Answer 36

The diagnosis can be deduced from the appearance on CT or MRI of a cerebellar cyst containing an enhancing nodular lesion on its wall

Question 37

Angio of HB

Answer 37

The angiographic picture is also characteristic: a cluster of small vessels forming a mass 1 .0 to 2.0 em in diameter

Question 38

Hemangioblastomas of the spinal cord are frequently associated with a _________ (greater than 70 percent of cases);

Answer 38

syringomyelic lesion

Question 39

four types of pineal tumors

Answer 39

germinoma, nongerminatous germ cell tumors, pinealoma (pineocytoma, atypical pineocytoma, and pineoblastoma), and a glioma originating in astroglial cells of the pineal body

Question 40

Of the four groups of pineal tumors, approximately 50 percent are \_\_\_\_\_\_\_

Answer 40

germinomas

Question 41

Cytologically, the __________ is a moderately cellular tumor with none of the histologic attributes of anaplasia.

Answer 41

pineocytoma

Question 42

T or F, pineocytoma forms rosettes

Answer 42

The tumor cells tend to form circular arrangements, so-called pineocytomatous rosettes

Question 43

\_\_\_\_\_\_\_\_\_\_ are highly cellular and composed of small, undifferentiated cells bearing some resemblance to medulloblasts

Answer 43

Pineoblastomas

Question 44

In patients with a germ cell tumor, the CSF or serum may show elevations of ______ or \_\_\_\_\_\_\_\_

Answer 44

B-human choriogonadotropin or alpha-fetoprotein

Question 45

a tumor that occurs in the adrenal gland, retroperitoneal and thoracic sympathetic chain, internal auditory canal, and in the spinal cord

Answer 45

gangliocytoma,

Question 46

The importance of distinguishing this disease from other cerebellar tumors is its lack of growth potential and favorable prognosis

Answer 46

dysplastic gangliocytoma of the cerebellum

Question 47

Imaging of dysplastic gangliocytoma

Answer 47

indistinct mass of "tiger stripe" appearance as a result of alternating layers of dysmorphic cerebellar cells

Question 48

syndrome of multiple skin hamartomas and cancers of the gynecologic, breast, and thyroid glands (and which may include Lherrnitte-Duclos as a component).

Answer 48

Cowden syndrome

Question 49

This CSF-filled lesion, which is probably congenital, presents clinically at all ages but may become evident only in adult life, when it gives rise to symptoms of increased intracranial pressure and sometimes to focal cerebral or cerebellar signs, simulating intracranial neoplasm

Answer 49

Arachnoid cyst

Question 50

Tx of Arachnoid cysts

Answer 50

The treatment o f enlarging and symptomatic cysts i s marsupialization or, less preferably, b y shunting from the cyst to the subarachnoid space.

Question 51

bilateral acoustic neuromas are the hallmark of the genetically distinct \_\_\_\_\_\_\_\_\_\_\_\_\_, in which they practically always occur before the age of 21 and show a strong (autosomal dominant) heredity

Answer 51

neurofibromatosis type 2 (NF2)

Question 52

The contrast-enhanced CT will detect practically all vestibular schwannomas that are larger than ______ cm in diameter and project further than \_\_\_\_\_\_cm into the cerebellopontine angle

Answer 52

2.0 1.5

Question 53

Preferred Tx for Vestibular Schwannoma

Answer 53

microsurgical suboccipital transmeatal approach

Question 54

In Vestibular Schwannoma, If no attempt is to be made to save hearing, small tumors can be removed safely by the\_\_\_\_\_\_

Answer 54

translabyrinthine approach

Question 55

This i s a histologically benign epithelioid tumor, generally assumed to originate from cell rests (remnants of the Rathke pouch [or adenohypophyseal diverticulum] ) at the junction of the infundibular stem and pituitary gland.

Answer 55

Craniopharyngioma

Question 56

What differentiates Craniopharyngioma from Adamantinoma

Answer 56

If there are bridges between tumor cells, which have an epithelial origin, the tumor is classed as an adamantinoma

Question 57

The tumor is thought to be derived from minute clusters of nonchromaffin paraganglioma cells (glomus bodies) found mainly in the adventitia of the dome of the jugular bulb (glomus jugulare) immediately below the floor of the middle ear, as well as in multiple other sites in and around the temporal bone

Answer 57

G l o m us J u g u l a re Tu m o r

Question 58

derivation of glomus jugulare tumor

Answer 58

minute clusters of nonchromaffin paraganglioma cells (glomus bodies)

Question 59

Tx of glomus jugulare tumor

Answer 59

Treatment in the past has consisted of radical mastoidectomy and removal of as much tumor as possible, followed by radiation

Question 60

This i s a generally benign but potentially malignant tumor originating in a small aggregate of paraganglioma cells of neuroectodermal type

Answer 60

Ca rot i d B o d y Tu m o r ( P a ra g a n g l i o m a )

Question 61

Presentation of Carotid Body Tumor

Answer 61

The usual presentation is of a painless mass at the side of the neck below the angle of the jaw

Question 62

Carotid body tumor has been seen in combination with _____ and \_\_\_\_\_\_\_\_

Answer 62

von Recklinghausen neurofibromatosis type 1 (NF1) and in von Hippel-Lindau disease

Question 63

On the basis of conventional hematoxylin-eosinstaining methods, cells of the normal pituitary gland were for many years classified as chromophobe, acidophil, and basophil, these types being present in a ratio of \_\_\_\_\_\_

Answer 63

5:4: 1

Question 64

Hormonal tests for the detection of Pituitary Adenoma

Answer 64

Question 65

In Amenorrhea-Galactorrhea Syndrome, In general, the longer the duration of amenorrhea and the higher the serum prolactin level,\_\_\_\_\_\_\_\_\_

Answer 65

the larger the tumor (prolactinoma) .

Question 66

Males with prolactin-secreting tumors rarely have galactorrhea and usually present with a larger tumorand complaints such as \_\_\_\_\_, ______ and \_\_\_\_\_

Answer 66

headache, impotence, and visual abnormalities

Question 67

In normal persons, the serum prolactin rises markedly in response to the administrationof \_\_\_\_\_\_\_\_\_\_patients with a prolactin-secreting tumor fail to show such a response.

Answer 67

chlorpromazine or thyrotropin-releasing hormone (TRH);

Question 68

This disorder consists of acral growth and prognathism in combination with visceromegaly, headache, and several endocrine disorders (hypermetabolism, diabetes mellitus).

Answer 68

Acromegaly

Question 69

Dx of Acromegaly

Answer 69

finding of elevated serum GH values (0.10 ng/mL), and the failure of the serum GH concentration to rise in response to the administration of glucose or TRH

Question 70

reserved for cases that are caused by the excessive secretion of pituitary ACTH, which, in turn, causes adrenal hyperplasia; the usual basis is a pituitary adenoma.

Answer 70

Cushing dse

Question 71

refers to the effects of cortisol excess from any one of several sources--excessive administration of steroids (the most common cause), adenoma of the adrenal cortex, ACTH-producing bronchial carcinoma, and, very rarely, other carcinomas that produce ACTH.

Answer 71

Cushing syndrome

Question 72

Dx of Cushing's dse

Answer 72

made by demonstrating increased concentration of plasma and urinary cortisol; these levels are not suppressed by the administration of relatively small doses of dexamethasone (0.5 mg qid), but they are suppressed by high doses (8 mg daily).

Question 73

a nontumorous enlargement of the sella. This results from a defect in the dural diaphragm, which may occur without obvious cause or with states of raised intracranial pressure, such as pseudotumor cerebri

Answer 73

Empty Sella Syndrome

Question 74

TX of Prolactinoma

Answer 74

The administration of the dopamine agonist bromocriptine (which inhibits prolactin) in a beginning dosage of 0.5 to 1 .25 mg daily (taken with food) may be the only therapy needed for small or even large prolactinomas, and is a useful adjunct in the treatment of the amenorrhea-galactorrhea syndrome.

Question 75

Some cases of acromegaly also respond to the administration of bromocriptine but even better to \_\_\_\_\_\_\_\_\_an analogue of somatostatin

Answer 75

octreotide,

Question 76

In Acromegaly, Newer slow-release somatostatin analogues and long-acting dopamine agonists such as \_\_\_\_\_\_have been developed for use in patients who do not respond to the conventional agents

Answer 76

cabergoline

Question 77

approximately \_\_\_\_\_\_\_\_percent of GH-secreting tumors and prolactinomas will recur at 1 year. For this reason, incomplete removal or recurrence of the tumor (or tumors that are unresponsive to hormonal therapy) should be followed by radiation therapy

Answer 77

15

Question 78

It is characterized by the acute onset of severe headache that may be retro-orbital, frontal, bitemporal, or generalized ophthalmoplegia; bilateral visual loss; and in severe cases, drowsiness or coma, with either subarachnoid hemorrhage or pleocytosis and elevated protein in the CSF

Answer 78

Pituitary Apoplexy

Question 79

Pituitary apoplexy may threaten life unless the acute addisonian state is treated by\_\_\_\_\_\_

Answer 79

hydrocortisone

Question 80

Factors that may precipitate the necrosis or hemorrhage of a pituitary tumor

Answer 80

anticoagulation, pituitary function testing, radiation, bromocriptine treatment, and head trauma; most cases, however, occur spontaneously

Question 81

T or F, In Brainstem Glioma Patients whose motor sx appear prior to those whose illness begins with cranial nerve palsies survive longer

Answer 81

T

Question 82

In Brainstem Glioma, diffusely infiltrating tumors, usually showing an asymmetrical enlargement of the pons, have a poorer prognosis than the\_\_\_\_\_\_\_tumors, which tend to occur in the dorsal brainstem

Answer 82

focal or nodular

Question 83

Tx of diffuse BGG

Answer 83

The treatment of the diffuse infiltrative type is radiation, and if increased intracranial pressure develops as a result of hydrocephalus, ventricular shunting of CSF becomes necessary.

Question 84

This i s a soft, jelly-like, gray-pink growth that arises from remnants of the primitive notochord

Answer 84

Chordoma

Question 85

Usual locations of chordoma

Answer 85

It is located most often within the clivus (from dorsum sellae to foramen magnum) and in the sacrococcygeal region

Question 86

Histology of chordoma

Answer 86

The tumor is made up of cords or masses of large cells with granules of glycogen in their cytoplasm and often with multiple nuclei and intracellular mucoid material.

Question 87

Lesions that may present as IC and EC masses

Answer 87

chordoma, meningioma, neurofibroma, glomus jugulare tumor, and carcinoma of the sinuses or pharynx

Question 88

Tx of Chordoma

Answer 88

Treatment of the chordoma is surgical excision and radiation (focused radiation). This form of treatment has effected a 5-year survival without recurrence in approximately 80 percent of patients

Question 89

summary of paraneoplastic and asstd antibodies

Answer 89

Question 90

The most distinctive features of \_\_\_\_\_\_\_\_\_\_\_\_\_consists of a confusional-agitated state, memory defect (Korsakoff syndrome), seizures, sometimes focal, hallucinations, and dementia-singly or in various combinations and evolving subacutely

Answer 90

paraneoplastic limbic encephalitis

Question 91

Vertigo, nystagmus, ataxia, nausea and vomiting, and a variety of ocular and gaze palsies reflect a different process of\_\_\_\_\_\_\_

Answer 91

paraneoplastic brainstem encephalitis

Question 92

Most patients with small cell lung cancer and any of the types of paraneoplastic encephalomyelitis have been found to harbor circulating polyclonal IgG antibodies \_\_\_\_\_\_\_\_\_\_\_\_that bind to the nuclei of neurons

Answer 92

(an ti-Hu, or antineuronal an tibody type 1 )

Question 93

The \_\_\_\_\_\_\_disorders tend to be slower in evolution and less severe than the typical paraneoplastic neurologic syndromes.

Answer 93

anti-VGKC

Question 94

A special form o f paraneoplastic encephalitis presents itself as an acute or subacute psychiatric syndrome consisting of some combination of hallucinations, panic, delusions, and incoherence, coupled with seizures, memory disturbance and hypoventilation has been described by Vitaliani and colleagues in four women with ovarian teratoma.

Answer 94

Anti - N M DA E nc e p h a l it i s

Question 95

Ab subunit target of anti-NMDA R enceph

Answer 95

the antibody as the subunit of the NMDA receptor, NRl

Question 96

Paraneoplastic syndrome with brainstem signs, particularly loss of horizontal gaze, and facial and pharyngeal spasms or abdominal myoclonus. Associated with what cancer?

Answer 96

Prostate CA

Question 97

This is a distinctive syndrome that is associated in most cases with the anti-Hu antibody. As the illness progresses, all forms of sensation are greatly reduced, resulting in disabling ataxia and pseudoathetoid movements of the outstretched hands

Answer 97

Para neoplastic Sensory N e u ro n o pathy

Question 98

For many years, this disorder was considered t o b e quite uncommon, but it is perhaps the most characteristic of the paraneoplastic syndromes.

Answer 98

Pa ra neoplastic Cerebel l a r Degeneratio n

Question 99

Cells affected in Paraneoplastic cerebellar degeneration

Answer 99

Purkinje cells are affected prominently and all parts of the cerebellar cortex are involved

Question 100

Anti-Purkinje cell antibodies \_\_\_\_\_\_\_\_\_can be found in the sera of about half of patients with paraneoplastic cerebellar degeneration and in the large majority of those related to carcinoma of the breast or female genital tract, linking the clinical syndrome and this antibody closely.

Answer 100

(termed "anti-Yo")

Question 101

Mechanism of Anti-Yo

Answer 101

They bind to a C-myc protein that initiates a degeneration of Purkinje cells

Question 102

manifest by rigidity and intense stimulus-sensitive myoclonus in addition to the core features of opsoclonus and ataxia. What antibodies are present?

Answer 102

anti-Ri antibody

Question 103

Classification of Paraneoplastic Motor Neuron syndromes

Answer 103

(1) rapidly progressive amyotrophy and fasciculations with or without brisk reflexes-all of their 3 patients displayed anti-Hu antibodies, 2 with small cell lung cancer and 1 with prostate cancer; (2) a predominantly corticospinal syndrome that affected the oropharyngeal or limb musculature, without definite evidence of denervation, thus resembling primary lateral sclerosis-all were breast cancer patients and none showed antineuronal antibodies; and (3) a syndrome indistinguishable from ALS in 6 patients with breast or small cell lung cancer, Hodgkin disease, or ovarian cancer, none of whom had antineuronal antibodies.

Question 104

The \_\_\_\_\_\_\_\_\_antibodies cross-react with testicular antigens and a search for a testicular tumor is undertaken

Answer 104

anti-Ma

Question 105

The antibody to\_\_\_\_\_\_\_\_\_ is reported in some series to be second in frequency only to anti-Hu. Lung carcinoma has been the most common source

Answer 105

CRMP-5

Question 106

Types of RT induced injury

Answer 106

Three syndromes of radiation damage have been delineated: acute, early delayed, and late delayed, although these syndromes often blend into one another

Question 107

Accepted levels of large-field radiation are tolerated in amounts approaching 6,000cGy, provided it is given in small daily doses\_\_\_\_\_\_\_

Answer 107

(200 to 300 cGy) 5 days per week over a period of 6 weeks

Question 108

Imaging features of RT Induced injury

Answer 108

CT and MRI show a contrast-enhancing lesion, and by angiography there is an avascular mass. Small calcifications may appear many years after the radiation

Question 109

radiation necrosis from tumor and peritumor products, but \_\_\_\_\_\_is the most reliable way of differentiating the two, perhaps obviating the need for biopsy

Answer 109

PET

Question 110

Tx of RT induced injury

Answer 110

Treatment has consisted of the administration of corticosteroids, which may cause regression of symptoms and of edema surrounding the lesion

Question 111

migraine-like syndrome following cranial irradiation

Answer 111

SMART, for stroke-like migraine attacks after radiation therapy