Tumors Flashcards
Possible gene defect in familial schwannomatosis
Mutations in SMARCB1 gene on chrom 22
T or F
Schwannoma practically always arise fr vestibular rather than cochlear division of CN VIII, just within IAC
True
Contrast CT will detect all vestibular schwannomas that are larger than ____ cm in diam and project further than ____ cm into the CPA
2 cm
1.5 cm
Most sensitive test to check for acoustic schwannoma
Brainstem evoked auditory response
Treatment for vestibular schwannoma
Surgical excision (microsurgical suboccipital transmeatal approach or translabyrinthine approach) OR
Focused radiation
OR focused radiation with gamma knife or proton beam in cases of recurrent tumor OR
Bevacizumab to reduce size of tumor in NF2
Contents of suprasellar epidermoid cyst
Dark albuminous fluid
Cholesterol crystals
Calcium deposits
Sxs of children with craniopharyngioma
Visual loss Diabetes insipidus Adiposity Delayed physical and mental devt Headaches Vomiting
Sxs of adults with craniopharyngioma
Waning libido Amenorrhea Slight spastic weakness of one or both legs Headache w/o papilledema Failing vision Mental dullness Confusion
Purplish red, highly vascular tumor composed of large epitheloid cells, arranged in an alveolar pattern aand possessing an abundant capillary network
Glomus jugulare tumor
From minute clusters of nonchromaffin paraganglioma cells (glomus bodies) found mainly in adventitia of dome of jugular bulb immediately below the floor of middle ear, as well as in multiple other sites in and around the temporal bone
Glomus jugulare tumor
Fr small aggregate of paraganglioma cells of neuroectodermal type
Carotid body tumor or paraganglioma
Many times more frequent in individuals living at high altitudes
Carotid body tumor or paraganglioma
Source of TIA in 5-15% of 600 cases
Carotid body tumor or paraganglioma
paraganglioma has been seen in combination with the ff diseases
Von recklinghausen NF type 1
Von hippel-lindau dse
Adenomas of pituitary are most often composed of what cells?
Chromophobe cells (4-20x as common as acidophil cell adenomas)
Hormones produce by either a chromophobe or an acidophil cell
Prolactin
GH
TSH
Hormones produce by basophil cells
ACTH
LH
FSH
beta-lipotropin
Percentage of pituitary tumors in both men and women are... prolactin secreting? GH secreting? ACTH? Gonadotropins and TSH?
60-70%
10-15%
Smaller #
Rare
Differential diagnoses for bitemporal hemianopia with a normal size sella
Saccular aneurysm of circle of willis
Meningioma of tuberculum sellae
Multiple sclerosis
Eventration of a greatly hydrocephalic third ventricle
Caused by the excessive secretion of pituitary ACTH, which, in turn, causes adrenal hyperplasia; the usual basis is a pituitary adenoma
Cushing disease
Percent of GH secreting tumors and prolactinomas will recur at 1 year postoperatively
15%
Effects of cortisol excess fr any one of several sources — excessive admin of steroids ( most common), adenoma of adrenal cortex, ACTH- producing bronchia Ca
Cushing syndrome
Increased concentration of plasma and urinary cortisol; these levels are not suppressed by admin of relaticely small doses of dexamethasone 0.5mg QID, are suppressed by high doses at 8mg OD
Cushing disease
Low ACTH plus
High cortisol in blood plus
Increased free cortisol in urine plus
Nonsuppression of adrenal fxn after admin of high doses of dexamethasone
Cushing syndrome
New growth hormone receptor antagonist used to reduce many of the manifestations of acromegaly
Pegvisomont
Factors that may precipitate the necrosis or hemorrhage of a pituitary tumor
Anticoagulation Pituitary function test Radiation Bromocriptine tx Head trauma
Ischemic necrosis of the pituitary, without a tumor, followed by hypopituitarism, most common being in partum or postpartum period
Sheehan syndrome
In gliomas of the brainstem, particularly astrocytomas…
True or False?
Patients who had long tract signs as their initial manifestations survive longer than those whose illness begins with CN palsies
True
DDX for pontine astrocytoma
Pontine form of MS
Cavernous hemangioma
Brainstem encephalitis
Longer survival in adults with pontine glioma as compared to children, median # of months?
54 months
DDX for monocular blindness with proptosis
Glioma of optic nerve and chiasm
Medial sphenoid, olfactory groove, and intraorbital meningiomas
Soft, jelly-like, gray-pink growth that arises fr remnants of the primitive notochord;
Located most often within the clivus and in the sacrococcygeal region
Chordoma
Cords or masses of large cells with granules of glycogen in their cytoplasm and often with multiple nuclei and intracellular mucoid material
Chordoma
Neck pain radiating to vertex of the skull on neck flexion
Chordoma
One of the rare causes of syndromes involving multiple CNs or cauda equina
Chordoma
DDX of lesions that may present both as an intracranial and extracranial mass
Chordoma Meningioma Neurofibroma Glomus jugulare tumor Carcinoma of sinuses or pharynx Midline (Wegener) granulomas Histiocytosis Erdheim- chester disease Sarcoidosis Chondrosarcoma of clivus
Pain in suboccipital or posterior cervical region mostly on the side of the tumor
Around the clock paralysis
Atrophic weakness of the hand or forearm or even intercostal ms with diminished tendon reflexes below level of tumor
Sensation of intense cold in neck and shoulders
Bands of hyperesthesia around the neck and back of the head
Segmental bibrachial sensory loss
Lhermitte sign
Tumors of the foramen magnum
Systemic malignant tumors that metastasize to basal skull bones
Prostate
Lung
Breast
Reliable marker for lung cancer in pxs with sensory neuronopathy
Anti-Hu antibody
paraneoplastic syndrome that is due to antibodies directed against the metabotropic glutamate receptor, mGluR5
Ophelia syndrome
Diagnostic triad of paraneoplastic retinopathy
Photosensitivity
Ring scotomas
Attenuation of retinal arterioles
Autoantibody involved in Stiff Person Syndrome (but not to stiff woman syndrome)
Anti-glutamic acid decarboxylase (GAD)
Autoantibody involved in choree fibrillaire of Morvan
Anti- VGKC
Autoantibody involved in LEMS
Anti-VGCC
Autoantibody involved in basal ganglionic syndromes particularly chorea
Anti-Hu
Anti-CRMP-5
