TUMORS2 Flashcards
Almost ______ of all leukemic patients have evidence of diffuse infiltration of the leptomeninges and cranial and spinal nerve roots at autopsy
one-third
The incidence is greater in acute than in chronic leukemia and greater in ______ than in myelocytic leukemia
lymphocytic
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)
acute lymphocytic (lymphoblastic) leukemia
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
pial disease
_______ and __________ given intrathecally or intravenously; has been effective in the prevention and treatment of meningeal involvement in childhood leukemia
Cranial radiation, combined with methotrexate
Cx of chemoRT after tx of meningeal involvement in childhood leukemia
necrotizing leukoencephalopathy within several days to months after the last administration of methotrexate and several months after completion of radiotherapy
MRI findings of necrotizing leukoenceph vs radiation necrosis
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
__________ 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
Extradural compression
In the rare cases of meningeal involvement with _______, there may be an eosinophilic pleocytosis
Hodgkin lymphoma
These are malignant tumors composed of cells derived from connective tissue elements (fibroblasts, rhabdomyocytes, lipocytes, osteoblasts, smooth muscle cells
S a rc o m a s of t h e B ra i n
_______ have occurred after radiation of pituitary adenomas and osteogenic sarcoma after other types of radiation, all localized to bone or meninges.
Fibrosarcomas
_________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
Medulloblastoma
Origin of Medulloblastoma
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
Certain molecular genetic similarities relate the medulloblastoma to _________, ____________ and, rarely, to autosomal dominant diseases such as ________
retinoblastomas and pineal cell tumors, nevoid basal cell carcinoma.
Chromosomal studies of medulloblastomas reveal a deletion on the distal part of chromosome _______ distal to the p53 region.
1 7
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
Gorlin syndrome Turcot syndrome
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)
MYCN (N-MYC)
In Medulloblastoma, The majority of the patients are children ______years of age, and males outnumber females_______ in most reported series
4 to 8 3:2 or 3:1
In Medulloblastoma,
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
________ 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
Maximal resection chemotherapy and radiotherapy
In Medulloblastoma, The combination of surgery, radiation of the entire neuraxis, and chemotherapy permits a 5-year survival in more than______ percent of cases
80
In Medulloblastoma, The presence of__________ (i.e., connective tissue formations) is associated with a better prognosis independent of the type or treatment
desmoplastic features
In Medulloblastoma, Any of the features of _______, ________, _______ greatly reduce the period of survival.
brainstem invasion, spinal subarachnoid metastases, incomplete removal, and very early age of onset (younger than 3 years)
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.
Neuroblastoma
Paraneoplastic complication of NB
polymyoclonus with opsoclonus and ataxia
T or F A rare form of neuroblastic medulloblastoma in adults tends to be more benign
T
Poor prognosticating factors in NB
- loss of heterozygosity of certain sites on chromosomes 1 and 11 2. MYCN amplification or overexpression 3. polymorphisms in chromosome 6p`
Treatment of NB low intermediate high
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
SR of patients with NB
survival rates exceeding 90 percent, but the highest risk group has a 30 percent survival rate
This proves to be one of the most frequent extracranial malignant tumors of infancy and childhood
RB
It is a small cell tumor with neurofibrils and, like neuroblastoma, has a tendency to form rosettes.
RB
Whereas the supratentorial ependymoma occurs at all ages, _____ ependymomas appear mostly in childhood.
fourth ventricular
The most anaplastic form of ependymoma is the _______________, a highly aggressive tumor that falls within the spectrum of primitive neuroectodermal tumors
ependymoblastoma
Locations of choroid plexus papilloma
They arise mainly in the lateral and fourth ventricles, occasionally in the third.
Molecules associated with tumor induction in choroid papilloma
T (tumor) antigen of the SV40 virus is possibly involved in tumor induction
MRI appearance of hemangioblastoma
The diagnosis can be deduced from the appearance on CT or MRI of a cerebellar cyst containing an enhancing nodular lesion on its wall
Angio of HB
The angiographic picture is also characteristic: a cluster of small vessels forming a mass 1 .0 to 2.0 em in diameter
Hemangioblastomas of the spinal cord are frequently associated with a _________ (greater than 70 percent of cases);
syringomyelic lesion
four types of pineal tumors
germinoma, nongerminatous germ cell tumors, pinealoma (pineocytoma, atypical pineocytoma, and pineoblastoma), and a glioma originating in astroglial cells of the pineal body
Of the four groups of pineal tumors, approximately 50 percent are _______
germinomas
Cytologically, the __________ is a moderately cellular tumor with none of the histologic attributes of anaplasia.
pineocytoma
T or F, pineocytoma forms rosettes
The tumor cells tend to form circular arrangements, so-called pineocytomatous rosettes
__________ are highly cellular and composed of small, undifferentiated cells bearing some resemblance to medulloblasts
Pineoblastomas
In patients with a germ cell tumor, the CSF or serum may show elevations of ______ or ________
B-human choriogonadotropin or alpha-fetoprotein
a tumor that occurs in the adrenal gland, retroperitoneal and thoracic sympathetic chain, internal auditory canal, and in the spinal cord
gangliocytoma,
The importance of distinguishing this disease from other cerebellar tumors is its lack of growth potential and favorable prognosis
dysplastic gangliocytoma of the cerebellum
Imaging of dysplastic gangliocytoma
indistinct mass of “tiger stripe” appearance as a result of alternating layers of dysmorphic cerebellar cells
syndrome of multiple skin hamartomas and cancers of the gynecologic, breast, and thyroid glands (and which may include Lherrnitte-Duclos as a component).
Cowden syndrome
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
Arachnoid cyst
Tx of Arachnoid cysts
The treatment o f enlarging and symptomatic cysts i s marsupialization or, less preferably, b y shunting from the cyst to the subarachnoid space.
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
neurofibromatosis type 2 (NF2)
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
2.0 1.5
Preferred Tx for Vestibular Schwannoma
microsurgical suboccipital transmeatal approach
In Vestibular Schwannoma, If no attempt is to be made to save hearing, small tumors can be removed safely by the______
translabyrinthine approach
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.
Craniopharyngioma
What differentiates Craniopharyngioma from Adamantinoma
If there are bridges between tumor cells, which have an epithelial origin, the tumor is classed as an adamantinoma
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
G l o m us J u g u l a re Tu m o r
derivation of glomus jugulare tumor
minute clusters of nonchromaffin paraganglioma cells (glomus bodies)
Tx of glomus jugulare tumor
Treatment in the past has consisted of radical mastoidectomy and removal of as much tumor as possible, followed by radiation
This i s a generally benign but potentially malignant tumor originating in a small aggregate of paraganglioma cells of neuroectodermal type
Ca rot i d B o d y Tu m o r ( P a ra g a n g l i o m a )
Presentation of Carotid Body Tumor
The usual presentation is of a painless mass at the side of the neck below the angle of the jaw
Carotid body tumor has been seen in combination with _____ and ________
von Recklinghausen neurofibromatosis type 1 (NF1) and in von Hippel-Lindau disease
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 ______
5:4: 1
Hormonal tests for the detection of Pituitary Adenoma
In Amenorrhea-Galactorrhea Syndrome,
In general, the longer the duration
of amenorrhea and the higher the serum prolactin
level,_________
the larger the tumor (prolactinoma) .
Males with prolactin-secreting tumors rarely have
galactorrhea and usually present with a larger tumorand complaints such as _____, ______ and _____
headache, impotence, and visual
abnormalities
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.
chlorpromazine or thyrotropin-releasing hormone
(TRH);
This disorder consists of acral growth
and prognathism in combination with visceromegaly,
headache, and several endocrine disorders (hypermetabolism,
diabetes mellitus).
Acromegaly
Dx of Acromegaly
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
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.
Cushing dse
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.
Cushing syndrome
Dx of Cushing’s dse
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).
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
Empty Sella Syndrome
TX of Prolactinoma
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.
Some cases of acromegaly also respond to the administration of bromocriptine but even better to _________an analogue of somatostatin
octreotide,
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
cabergoline
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
15
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
Pituitary Apoplexy
Pituitary apoplexy may threaten life unless the acute
addisonian state is treated by______
hydrocortisone
Factors that may precipitate the necrosis or hemorrhage
of a pituitary tumor
anticoagulation, pituitary function
testing, radiation, bromocriptine treatment, and head
trauma; most cases, however, occur spontaneously
T or F,
In Brainstem Glioma
Patients whose motor sx appear prior to
those whose illness begins with cranial nerve palsies survive longer
T
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
focal or nodular
Tx of diffuse BGG
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.
This i s a soft, jelly-like, gray-pink growth that arises from
remnants of the primitive notochord
Chordoma
Usual locations of chordoma
It is located most often within the clivus (from dorsum sellae to foramen magnum) and in the sacrococcygeal region
Histology of chordoma
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.
Lesions that may present as IC and EC masses
chordoma, meningioma, neurofibroma, glomus jugulare
tumor, and carcinoma of the sinuses or pharynx
Tx of Chordoma
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
summary of paraneoplastic and asstd antibodies
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
paraneoplastic limbic
encephalitis
Vertigo, nystagmus, ataxia, nausea and vomiting,
and a variety of ocular and gaze palsies reflect a different
process of_______
paraneoplastic brainstem encephalitis
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
(an ti-Hu, or antineuronal an tibody type 1 )
The _______disorders tend to be slower in
evolution and less severe than the typical paraneoplastic
neurologic syndromes.
anti-VGKC
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.
Anti - N M DA E nc e p h a l it i s
Ab subunit target of anti-NMDA R enceph
the antibody as the subunit of the NMDA receptor, NRl
Paraneoplastic syndrome with
brainstem signs, particularly loss of horizontal
gaze, and facial and pharyngeal spasms or abdominal
myoclonus.
Associated with what cancer?
Prostate CA
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
Para neoplastic Sensory N e u ro n o pathy
For many years, this disorder was considered t o b e quite
uncommon, but it is perhaps the most characteristic of
the paraneoplastic syndromes.
Pa ra neoplastic Cerebel l a r Degeneratio n
Cells affected in Paraneoplastic cerebellar degeneration
Purkinje cells are affected prominently and all parts of
the cerebellar cortex are involved
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.
(termed “anti-Yo”)
Mechanism of Anti-Yo
They bind to a C-myc protein
that initiates a degeneration of Purkinje cells
manifest by rigidity and intense stimulus-sensitive myoclonus in addition to the core features of opsoclonus and ataxia. What antibodies are present?
anti-Ri antibody
Classification of Paraneoplastic Motor Neuron syndromes
(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.
The _________antibodies cross-react with testicular antigens and a search for a testicular tumor is undertaken
anti-Ma
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
CRMP-5
Types of RT induced injury
Three syndromes of radiation damage have
been delineated: acute, early delayed, and late delayed,
although these syndromes often blend into one another
Accepted levels of large-field
radiation are tolerated in amounts approaching 6,000cGy, provided it is given in small daily doses_______
(200 to 300
cGy) 5 days per week over a period of 6 weeks
Imaging features of RT Induced injury
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
radiation necrosis from tumor and peritumor products, but ______is the most reliable way of differentiating the two, perhaps obviating the need for biopsy
PET
Tx of RT induced injury
Treatment has consisted of the administration of
corticosteroids, which may cause regression of symptoms
and of edema surrounding the lesion
migraine-like syndrome following cranial irradiation
SMART, for stroke-like migraine attacks after radiation therapy
