What feature is similar about Warthin's tumor, pleomorphic adenoma, and mucoepidermoid carcinoma?
all commonly localize to the parotid gland
papillary cystadenoma lymphomatosum and adenolymphoma other names for what? Why is it helpful to remember this?
Warthin's tumor - useful because cystic glandular benign tumor (cystadenoma) with lymph node-like stroma consisting of abundant lymphocytes and germinal centers (lymphomatosum ). Cystic spaces are surrounded by two uniform rows of cells with centrally placed pyknotic nuclei papillary - b/c epithelium surrounding cystic spaces exhibits "papillary infolding" adenolymphoma - useful to remember because neoplasm of glandular tissue (adenoma) with lymph-node like structures (lymphoma) *only type of salivary gland tumor more common in men than women, 15% of cases are bilateral
Rough whitish patch on the lateral tongue, usually arising in immunocompromised Pts, cannot be scraped off. What is it? What causes it? Is it cancerous?
Hairy leukoplakia Caused by EBV which induces squamous cell hyperplasia (not dysplasia!) not pre-malignant
Painful superficial ulceration of the oral mucosa that is characterized a greyish base surrounded by erythema What condition is this? What causes it?
Aphthous ulceration arises in relation to stress, spontaneously resolves
vesicles in the oral mucosa that rupture and result in shallow painful red ulcers. Usually seen in children. What is this? What causes it?
Primary HSV-1 infection Lesions heal but virus remains dormant in trigeminal nerve ganglion
Vesicles that rupture and result in shallow painful red ulcers - often occurring on the lips. What is it what causes it?
Reactivation of HSV-1 inefection that caused by stress or exposure to sunlight
How can you differentiate aphthous ulcers from primary HSV-1 infections?
primary HSV-1 doesn't have grey base of lesion and usually occurs in childhoord. HSV-1 reactivations don't have grey base and also occur on the lips
syndrome marked by recurrent aphthous ulcers, genital ulcers and uveitis What is it? What causes symptoms?
Bechet syndrome small vessel immune-complex vasculitis
easily scraped away white deposit on tongue seen in immunocompromised states
Oral candidiasis aka thrush
reddish plaque on tongue that cannot be scraped away . What is this? What is it a strong indicator of? Is it cancerous, pre-cancerous or benign?
Erythroplakia squamous cell dysplasia of the oral mucosa per-cancerous ==> can lead to squamous cell carcinoma Alcohol and tobacco are biggest risk factors
Protrusion of edematous, inflammed mucosa that usually arises secondary to repeated bouts of rhinitis but also occurs in cystic fibrosis and aspirin-intolerant asthma.
Protrusion of edematous, inflammed mucosa in a child should be checked for what? In adults?
cystic fibrosis Aspirin intolerant asthma
What is the most common cause of a malignant turmor of the nasopharyngeal epithelium? Who is most susceptible?
Nasopharyngeal carcinom - caused by EBV infection African CHILDREN or Chinese ADULTS
Pt. 16 YO male presents with recurrent epistaxis - what could be the cause?
Nasopharyngeal angioblastoma benign but locally aggressive vascular tumor that grows in the back of the nasal cavity (consists of large blood vessels and fibrous CT). Most commonly affects adolescent males. Usually presents with one-sided nasal obstruction and recurrent bleeding.
45 YO chinese pt. presents with swollen cervical lymph node, blood in saliva, bloody discharge from the nose, nasal congestion, hearing loss, frequent ear infections, headaches. What is a likely diagnosis? What might have caused this?
nasopharyngeal carcinoma secondary to EBV infection
biopsy of a nasopharyngeal carcinoma would likely show what two histological features?
pleomorphic keratin-positive (poorly differentiated) epithelial cells Background of lymphocytes
Most common cause of acute epiglottitis?
H influenza type B (especially among non-immunized)
inflamation of the epiglottis that results in high fever, sore throat, drooping with dysphagia, muffled voice, inspiratory stridow, and risk of airway obstruction
What is croup? What is the most common cause?
inflammation of the upper airway (aka laryngotracheaobronchitis) parainfluenza virus
Pt. is a singer who presents with hoarseness that resolves with resting of voice - what might they have?
vocal cord nodule - nodules on true vocal cords that develop from overuse, usually bilaterally, composed of degenerative(mixoid) CT. not a laryngeal papilloma (also has hoarseness) because resolves with non-use
what causes benign tumors on the vocal cords that present with hoarseness and does not get better with resting voice?
HPV 6 or 11 laryngeal papilloma
T/F layngeal papillomas are usually multiple in children and single in adults
T/F laryngeal papillomas are typically associated with epithelial dysplasia
False - not typically, only rarely pre-cancerous! But does occur - HPV 6 , 11 "Low risk" for cancer -- though can lead to laryngeal carcinoma in rare cases
Most common causes of epithelial dysplasia on true vocal cords that transisions to squamous cell carcinoma of the larynx (laryngeal carcinoma)?
Alcohol and tobacco
benign mobile painless and circumscribed mass in the parotid gland with high recurrence rate after surgical excision
in rare cases pleomorphic adenomas can become malignant resulting in parotid adenocarcinoma. What clinical symptom would be indicative of possible malignancy and why?
diffuse unilateral upper and lower facial pain -- facial nerve runs through parotid gland
Pt presents with bilaterally inflammed parotid glands, testicular pain, feeling of bloating after eating with diffuse left upper abdominal pain that radiates to the back, painful headache, fever, sensitivity to light, nausea and vomiting. Labs show high serum amylase. What do they have? What is the greatest risk of complications in teenagers?
Mumps virus infection with orchitis, pancreatitis and aseptic meningitis sterility
How are neural tube defects usually detected during pregnancy?
elevated AFP levels in amniotic fluid or maternal blood
Congenital failure of cerebellar vermis to develop resulting in massively dilated 4th ventricle very diminished or absent cerebellum; often accompanied by hydrocephalus.
Congenital downward displacement of cerebellar vermis and tonsils through the foramen magnum that can obstruct CSF flow and cause hydrocephalus - very commonly associated with meningomyelocele
Arnold-Chiari malformation Type II meningomyelocele is protrusion of meninges and spinal cord in spinal bifida
cystic degeneration of the spinal cord caused by the presence of a fluid filled cavity that usually occurs between C8-T1 and results in sensory loss of pain and temperature with sparing of fine touch and position sense in the upper exremities that follows a "cape-like" distribution. As fluid cavity expands, progresses to involve muscle atrophy and weakness with decreased muscle tone and impaired reflexes, ptosis, miosis, and anhidrosis
T/F polio virus damages the anterior horn of the spinal cord producing lower motor neuron lesion symptoms
true - syndrome called poliomyelitis flaccid paralysis, muscle atrophy, fasciculations, weakness, decreased muscle tone, impaired reflexes, negative babinski sign
What are symptoms of a lower motor neuron lesion?
flaccid paralysis, muscle atrophy, fasciculations, weakness, decreased muscle tone, impaired reflexes, negative babinski sign
What are symptoms of an upper motor neuron lesion?
result from damage to descending motor systems; include paralysis, spasticity, and a positive Babinski sign.
Inherited degeneration of the anterior motor horn; autosomal recessive, presents as "floppy baby", usually fatal within several years of life
Degenerative disorder of upper and lower motor neurons of the corticospinal tract that typically appears in middle age adults, marked by early atrophy and weakness of the hands and overall lack of sensory impairment.
ALS familial cases have SOD1 mutation
What differentiates ALS from Syringomyelia?
lack of sensory impairment in ALS
Autosomal recessive degenerative disorder of the spinal cord and cerebellum that presents in early childhood and leads to loss of vibratory sense and proprioception, muscle weakness in the lower extremities, loss of deep tendon reflexes and ataxia
What is the genetic defect responsible for Freidrich Ataxia
autosomal recessive mutation of the frataxin gene due to the expansion of an unstable trinucleotide repeat (GAA)
What is frataxin?
protein essential for mitochondrial regulation of iron; loss results in iron buildup and free radical damage
What serious associated medical condition accompanies friedrich ataxia?
T/F ALS usually appears in mid-adulthood and is typically not caused a genetically inherited mutation
True -- SOD1 mutation is present in a fraction of familial cases but these are a vast minority of total ALS cases
What is most common cause of bacterial meningitis in adults and elderly?
What is most common cause of bacterial meningitis in children and teenagers?
Niseria meningitidis - enters through nasopharynx and then gets to blood then from blood gets to the meninges
What is the most common cause of bacterial meningitis in neonates?
Group B strep, E coli, Listeria monocytogenes
What is the most common cause of bacterial meningitis in infants?
H influenza (particularly in non-vaccinated instants)
What is a viral cause of meningitis?
Enteroviruses (e.g. coxsackievirus) are most common causes (fecal oral transmission, particularly affects children)
What is the most common source of group B strep and E coli infection that leads to meningitis in neonates
exposure in vaginal canal during delivery group B strep is tested in 3rd trimester for prophylaxis
How do children and teenagers get meningitis from n minigititis?
enters through nasopharynx and then gets to blood then from blood gets to the meninges
Common cause of meningitis in immunocompromised patients?
fungal infections of criptoccocus and aspergillus
What is the classic triad of symptoms in meningitis?
Headache, nuchal rigidity and fever -- other common symptoms are photo-phobia, vomiting, and altered mental status
rapidly progressive gangrenous cellulitis of the soft tissues of the neck and floor of the mouth that results in swelling of the tongue, neck pain, and breathing problems - commonly associated with dental procedures - culture produces mixoid aeorobic and non-aerobic bacterial flora
Ludwig's angina (cellulitis = spreading bacterial infection under skin; angina = strangling)
What is Gottron's sign? What disease is it used to detect?
Descrete erythematous, scaly eruptions occurring in symmetric fashion overlying the metacarpal and interphalangeal joints Dermatomyositis
What is xerostomia? keratoconjunctivitis sicca? What disease are both of these found in?
dry mouth, dry eyes Sjogren syndrome
Chronic inflammatory disease of the salivary and lacrimal glands that may be restricted there or be part of a systemic collagen vascular disease
What happens to salivary and lacrimal glands as dissease course progresses?
Become atrophic with fibrosis and fatty prarenchymal infiltrates
Differential for bilateral parotid gland swelling includes?
Sjogren syndrome; Mumps virus infection; Warthin cysts (15% of cases bilateral)
focally ulcerated reddish lesion ~2mm - 1cm located on the gingiva of the mouth with highly vascularized granulation tissue
CSF findings from bacterial meningitis?
neutrophils with decreased CSF glucose
CSF findings from viral meningitis?
lymphocytes with normal CSF glucose
CSF findings from fungal meningitis?
lymphocytes with decreased CSF glucose
Most common cause of post-natal unilateral deafness from viral infection?
same as otitis interna - infection of inner ear and vestibular system commonly caused by viruses (e.g. mumps, rubella
Prenatal viral infection that causes bilateral deafness and permanant damage to the choclea and vestibular system
postnatal viral infection that causes unilateral deafness
Inflammation of the salivary gland usually due to an obstructing stone and subsequent staph aureus infection; usually unilateral
Tumor in the nasal cavity that can cause "sinus pressure". Marked by epithelial nests protruding into the submucosa with uniform proliferation and no evidence of cellular atypia.
sinonasal/nasolaryngeal inverted papilloma - caused by HPV 6,11 Inverted papilomas feature inversion of the epithelium into the underlying stroma encased by intact basement membane (so tumor can protrude into the nasal sinuses - think opposite of a polyp).
Most common infectious agent in acute suppurative tonsilitis?
consequence of chonric suppurative otidis media with rupture of typanic membrane
mass of accumulated keratin and squamous mucosa that can cause hearing loss and results from growth of squamous epithelium from the external ear through the ruptured ear drum into the middle ear
most clinically significant odontogenic tumor that benign tumor of odontogenic epithelium more commonly appearing in the lower jaw than the upper jaw. Rarely metastatic but progress slowly, resulting in lesions can cause severe abnormalities of the face and jaw
Hydrocephalus in a neonate associated with seizures and periventricular calcifications. Histology shows pink intranuclear/intracellular inclusions. What was the cause?
Vertical transmission of Cytomegalovirus accross the placenta in utero. TORCH = Toxoplasmosis, Rubella, Cytomegalovirus, Herpes simplex
"opportunistic" virus that causes progressive multifocal leukoencephalopathy in immunocompromised states (e.g. AIDS or organ transplant).
Most susceptible brain regions (and cells) to damage from ischemia
perkinje neurons of cerebellum and pryimidal neurons of Sommner's sector in hippocampus
can cause death secondary to truama resulting in diffuse lesions/petechia in white matter
fat emboli - primarily affect capillaries - high density of capillaries at white matter/grey matter interface
Favored sites of aneurysms and most common consequence
internal corotid, vertibral, basilar arteries thrombosis > hemorrhage /dissection
Watershed zone most commonly affected by deminished cerebral blood flow (e.g. prolonged hypotension)
overlap zone between terminal branches of middle cerebral and anterior cerebral (watershed zone) - results in parasaggital infarcts
most common cause of decline in mental status following TBI - produces "spheroids", parasagital white matter damage accompanied by multiple infarcts - due to sheer forces from anchoring to arachnoid villi
diffuse axonal injury
result of uncle transtentorial herniation
Most common cause of death from ALS
respiratory insufficiency ALS is a degenerative disease of motor neurons of the brain and spinal cord (atrophy of ventral roots, motor cortex, and motor nuclei in brainstem) that results in weakness and wasting of the muscles of the hands (can be unilateral) accompanied by fasciculations and pain from muscle wasting, rapid decline and death, usually in 6 years or less
Defining histological change in the brain from ALS
loss of motor neurons, atrophy of motor cortex, gliosis
inborn error of metabolism/lysosomal storage disorder caused by autosomal recessive mutation in lysosomal enzyme arylsulphatase. Causes accumulation of the cerebroside galactosyl sulphatide in white matter leading to astrogliosis, failure to thrive and death (~6 months) from respiratory insufficiency
multichromatic leukodistrophy - most common leukodystrophy accumulation of sufatides (breakdown product of myelin-sulfatides) in lysosomes and "metachromatic material" in white matter
x-linked inborn error of metabolism high levels of long-chain fatty acids in tissues and body fluids dysfunctional adrenal glands and bilaterally symetric demyelination, gliosis, and perivascular lymphocytic infiltrates Disease is _____ caused by ____
Adrenoleukodystrophy caused by defective peroxisomes
Fatal infantile disease associated with autosomal recessive diffeciency in galactocerebrocide beta-galactosidases
Krabbe disease is associated with what histological findings?
White matter disease, associated with perivascular accumulation of mononuclear and and multinucleated "globoid cells" containing undigested galactosylcerimide
Defective GFAP Rosenthal fibers
Accumulation of mucopolysaccarides
Cause of subacute sclerosing panencephalitis
reactivation of latent measles virus
Histologic features of subacute sclerosing panencephalopathy
patchy loss of myelin, prominant intranuclear inclusions in neurons and oligodendrocytes, marked gliosis affecting grey and white matter, perivascular lymphocytes and macrophages
What causes subacute slclerosing panencephalopathy? How is disease different in children vs adults?
reactivated measles virus In children time course is protracted and primarily affects cerebral grey matter in adults time course is more rapid
Fatal familial insomnia and spongiform encephalopathy are caused by ________
First signs of medulloblastoma
symptoms of hydrocephalus, truncal ataxia and wide based gate rapid and regionally infiltrative tumor of the cerebellum
symptoms of hydrocephalus in children
vomiting, sleepiness/lethargy, irritability, downward deviation of the eyes (also called "sunsetting"), and seizures
Midline cerebellar tumor characterized histologically by small hyperchromatic cells with neuroblastic rosettes
cell-markers (suggestive of meduloblastomas, ependoma or pineal parenchymal tumors) that rule out lymphoma, metastatic epithelial tumor or neuroectodermal malignancy
synaptophysin or nestin
hereditary disorder in which cerebellar hemangioblastomas are associated with retinal angiomas and other turmors like renal cell carcinoma
von-hippel lindau syndrome
Cause of von-hippel lindau syndrome
mutated VHL tumor suppressor gene
Clincal signs of Von-Hippel Lindau syndrome
headache, unsteadiness in walking (wide based gait), unilateral impaired coordinated movements in arm and leg (disdiodokinesia), intentional tremor
non-familial congenital disorder featuring facial "port wine stain" (nevus flammeus) and leptominingeal angiomas
Associated disease: highly vascularized cerebellar tumor that features endothelium lined cannals interspersed with "plump cells" that may secrete eyrethropoin and induce polycythemia
Von-Hippel Lindau syndrome
Relative likelihood of intracranial metastasis Colon cancer Breast cancer Lung cancer Melanoma Renal cancer
marker for melanoma used to diagnose brain metastasis
invasive brain tumor that exhibits vascular changes ("arteric obliterateration"), pallasading pleiomorphic, multinuclear tumor cells around patches of yellow necrosis, and redzones of hemorrhage
glioblastoma multiforme tumor cells comprised of astroyctes can present with "butterfly lesion", large tumor extending bridge of malignant cells across corpus collosum** **can result in ipsilateral symptoms to large tumor.
tumor with characteristic "epithelial appearance" may feature "perivascular pseudorosettes" of tumor cells surrounding blood vessels typically associated with central canal of spinal cord, walls of 4th ventricle, and often results in hydrocephalus
slow growing benign tumor that may displace brain tissue and erode contiguous bone of the skull. Does not infiltrate the brain.
Histologic presentation of a miningeoma
well circumscribed, firm, "bossylated" masses of variable size with whirled pattern of meningothelial cells
Solid cystic lesion displacing the region of the hypotalamus (located above stella turcica) that arises from the Rathke pouch and compresses the medial aspect of the optic chiasm resulting in bitemporal hemianopsia, headaches, and sometimes pituitary failure that can result in diabetes insipidus
Cranialpharyngioma Rathke pouch = part of embyronic nasopharynx that migrates cephalad to give rise to the anterior lobe of the hypothalamus
Most likely type of brain tumor in AIDS patient
primary deep cerebral (B-cell) lymphoma with bilateral perivascular distribution
ocular lesions from deposition of copper in the Descemet membrane of the iris
Disease marked by degeneration of corpus striatum resulting in extrapyramidal symptoms (lack of coordination, tremor), chronic hepatitis/cirrhosis, deposition of copper in the iris causing Keyser-Feischer ocular lesions
slow growing tumor arising in the white matter marked by a dense patterning of neoplastic cells with small rounded nuclei, pleomorphic features, and randomly distributed (radiographically observable) "Calcospherites"
Cause of syndrome defined by bilateral benign tumors of the 8th cranial nerve (acustic neuromas), which are commonly associated with meningeioms and gliomas
Neurofibromatosis type II deletion of NF2 gene
What differentiates neurofibromatosis type I vs type II?
Both have neurofibromas but only type 2 has acustic neuromas - intracranial shwannoma of the 8th cranial nerve
What is pseudotumor cerebri?
ideopathic intracranial hypertension (increased pressure without evidence of tumor/obstruction) [+] papilloedema [-] mental status changes [-] focal neurologic findings
Cause of compression of midbrain, CNIII, PCA, parasympathetic fibers that results in Duret hemorrhages, down and out dilation (mydriatic) with hemmoragic infarction of occipital lobe
Uncal herniation (medial temporal lobe pushes through tentorium cerebelli)
Coning of cerebellar tonsils that produces cardiorespiratory arrest
Duret hemorrhage and causes
small midbrain bleeds cause by downward movement of brainstem or compression of midbrain
most common cause of laryngotracheobronchitis in children
parainfluenza virus ==> causes a type of laryngotracheobronchitis called "Croup"