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esophageal pathology
GI
73
Medical
Graduate
08/19/2010

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Term
what is dysphasia? odynophasia?
Definition
dysphasia is difficulty swallowing, odynophasia is painful swallowing
Term
what is nutcracker esophagus?
Definition
a lack of coordination in the muscular layers of the esophagus leading to short lived obstruction
Term
what can a diffuse esophageal spasm lead to?
Definition
a diffuse esophageal spasm can lead to functional obstruction and pseudodiverticula
Term
what is a zenker diverticulum? what is usually the cause?
Definition
a diverticulum above the upper esophageal sphincter that may grow large and trap food. zenker's diverticuli are usually due to points of weakness in the wall
Term
what is a tracker diverticulum? what is usually the cause?
Definition
a diverticulum near the midpoint of the esophagus. tracker diverticuli are usually due to inflammation and traction
Term
what is a epiphrenic diverticulum? what is usually the cause?
Definition
a diverticulum above the lower esophageal sphincter. epiphrenic diveriticuli are usually due to complications including pneumonia, perforation, mediastinitis and carcinoma
Term
what characterizes esophageal stenosis? can it be congential? what is is usually due to?
Definition
a narrowing of the lumen due to fibrous thickening of the submucosa and atrophy of the muscularis propria and epithelial damage. it can be congenital but is usually due to reflux, caustic material or irradiation. it results in a gradual narrowing, causing change in dietary habits from ingesting solids to liquids
Term
what is an esophageal web? who commonly gets them? what is associated with their incidence?
Definition
an uncommon ledge-like protrusion of mucosa, usually seen in women >40 and may be associated with GE reflux, graft v host disease, or blistering skin diseases
Term
what is *plummer-vinson syndrome*?
Definition
plummer-vinson syndrome includes upper esophageal webs, iron deficiency anemia, glossitis, and cheilosis
Term
where are esophageal webs most common? how do they appear? what are they composed of? what can they cause?
Definition
esophageal webs are most common in the upper esophagus and are semicircular lesions protruding < 5 mm into the lumen. they are composed of fibrovascular connective tissue and overlying epithelium. esophageal webs can cause dysphagia
Term
what are schatzki rings?
Definition
esophageal rings that are similar to webs, but circumferential and thicker
Term
what are the 2 types of schatzki rings?
Definition
A rings: in distal esophagus, above the GE junction and covered by squamous mucosa and B rings: may have gastric cardia-type mucosa on their undersurface
Term
what is achalasia?
Definition
achalasia is increased tone of the lower esophageal sphincter (LES), which leads to decreased relaxation of the LES and aperistalsis of the esophagus
Term
what is primary achalasia?
Definition
primary achalasia is the failure of the distal esophageal inhibitor neurons, possibly due to degenerative changes in neural innervation
Term
what specific kind of infection can lead to achalasia?
Definition
chagas disease, or infection by trypanosoma cruzi can lead to destruction of the myenteric plexus, which then leads to failure of peristalsis and esophageal dilatation
Term
what are some chronic diseases that can lead to achalasia-like symptoms?
Definition
DM, malignancy, and amyloidosis
Term
what are symptoms of achalasia?
Definition
dysphagia, vomiting, some chest pain
Term
what is a common esophageal laceration?
Definition
the mallory-weiss tear, which is a *longitudinal (vertical)* tear at the gastroesophageal junction - often due to retching associated w/alcohol intoxication (an alcoholic w/cirrhosis can die of bleeding out this way). this may cross into the GE junction, and can be lethal - though healing is usually the outcome
Term
what is boerhaave syndrome?
Definition
a distal esophageal rupture, usually the lower 1/3 in the L posterolateral region, accounting for 15% of esophageal ruptures
Term
what is boerhaave syndrome associated with? what causes it?
Definition
boerhaave syndrome is associated with mediastinitis (due to esophageal contents leaking out) and a high mortality rate. usually boerhaave syndrome occurs from a sudden increase in intraluminal pressure - often from violent vomiting following heavy food or alcohol intake
Term
why is the esophagus more vulnerable to rupture?
Definition
the esophagus lacks a serosal layer
Term
do both the mallory weiss and boerhaave tears go all the way through the esophageal wall?
Definition
only the boerhaave tear typically goes through the entire wall
Term
what can cause esophagitis?
Definition
alcohol, acid/alkali, medications, hot fluids, heavy smoking, radiation, chemo, and infections
Term
what characterizes esophagitis?
Definition
infiltration of neutrophils, wall necrosis, ulceration, granulation tissue, and possible fibrosis
Term
what is the *most common cause* of infectious esophagitis?
Definition
candidiasis, due to its adherent soft white pseudomembranes
Term
can herpes cause esophagitis?
Definition
yes, herpes can cause *round punched out ulcers and nuclear viral inclusions
Term
can CMV cause esophagitis?
Definition
yes, CMV can cause shallower, linear ulcerations w/nuclear *and cytoplasmic inclusions in capillary endothelium and stromal cells (cells are larger - 'mega')
Term
what is reflux esophagitis? what are contributing factors?
Definition
reflux of acid injuring the mucosa of stratified squamous epithelium (which is sensitive to acid) - giving rise to GERD. contributing factors include decreased LES tone, smoking, alcohol, obesity, pregnancy, and increased gastric volume (things that increase the intraluminal stomach pressure). reflux esophagitis is kind of the opposite of achalasia
Term
what are morphologic changes associated with reflux esophagitis?
Definition
hyperemia, infiltration by eosinophils and neutrophils and basal zone hyperplasia w/elongation of the lamina propria papillae
Term
who is GERD most commonly seen in?
Definition
adults over 40 - but it can be seen in children/infants
Term
what are the most common symptoms of GERD?
Definition
dysphagia and heartburn, sometimes severe chest pain (mimics an MI)
Term
what are common complications of GERD?
Definition
ulceration, barrett esophagus and stricture development
Term
what is eosinophilic esophagitis? are there other common co-presentations?
Definition
a marked increase in intraepithelial eosinophils in larger numbers than would be seen in reflux esophagitis. other common co-presentations are atopic dermatitis, allergic rhinitis, asthma, or peripheral eosinophilia (overall allergic asthmatic kinds of people). pts w/this may have significant dietary restrictions
Term
what is *barrett esophagus*? who does it most commonly affect?
Definition
a complication of GERD, where intestinal metaplasia occurs in the esophageal squamous epithelium (becomes adenomatous and columnar w/goblet cells). barrett esophagus most commonly affects white males between 40-60 and is associated with an approx 30x greater risk of developing adenocarcinoma. incidence is rising.
Term
what is the major risk with barrett esophagus?
Definition
the metaplasic component can give rise to a dysplastic component and then to well differentiated and finally poorly differentiated adenocarcinoma
Term
how is barrett esophagus diagnosed?
Definition
endoscopy/biopsy
Term
how does barrett esophagus present?
Definition
barrett esophagus presents as patches of red velvety mucosa that alternate with smooth pale squamous mucosa and light brown columnar gastric mucosa
Term
what is considered a long segment of barrett esophagus? short?
Definition
long segment: 3 cm or more
short segment: less than 3 cm
Term
what needs to be established above the gastroesophageal junction to dx barrett esophagus?
Definition
metaplastic epithelium
Term
what do metaplastic columnar cells contain?
Definition
goblet cells, non-goblet mucinous columnar cells, enterocytes, paneth cells and multilayered epithelium (Me)
Term
what is a sensitive and specific marker for GERD?
Definition
Me (multilayered epithelium)
Term
does multilayered epithelium (Me) have squamous or columnar differentiation?
Definition
both
Term
how is dysplasia defined?
Definition
increased levels of epithelial proliferation, either low/high grade
Term
is dysplasia detectable under endoscopy?
Definition
not generally
Term
how do dysplastic esophageal cells appear?
Definition
crowded glands w/abnormal glandular architecture, atypical mitosis and a high N-C ratio
Term
what is the most life threatening esophageal pathology associated with alcoholics?
Definition
esophageal varices
Term
what are esophageal varices composed of?
Definition
porto-systemic shunts w/congestion of collateral blood vessels - some of which are present in the esophageal submucosa.
Term
what diseases lead to the porto-systemic shunts seen in esophageal varices? does the primary causative disease depend on location in the world?
Definition
in western countries these shunts are usually due to cirrhosis, however worldwide, hepatic schistosomiasis is the second most common cause of varices
Term
what about esophageal varices makes them so life-threatening? how?
Definition
the potential for hemorrhage. vomiting can lead to erosion of the mucosa and with pressure from increasingly dilated veins, bleeding can be massive.
Term
are esophageal varices symptomatic in their early phase?
Definition
not usually
Term
how do esophageal varices appear?
Definition
as tortuous dilated veins in the submucosa of the distal esophagus and proximal stomach
Term
can esophageal varices be detected?
Definition
yes, via venogram
Term
why are esophageal varices difficult to detect in a surgical or postmortem specimen?
Definition
esophageal varices may be difficult to detect due to a lack of blood pressure - in a living state, the blood pressure forces the submucosal vessel closer to the surface, making them more prone to injury and bleeding
Term
what are the most common esophageal tumors?
Definition
squamous cell carcinoma and adenocarcinoma
Term
who are esophageal squamous cell carcinomas more common in? what are risk factors?
Definition
males over 45 in rural/underdeveloped countries (high incidence in iran, central china, hong kong, brazil, south africa). risk factors include *alcohol, *tobacco, plummer-vinson syndrome, achalasia, caustic esophageal injury, previous radiation therapy to the mediastium, nutritional deficiency, nitrosamines, *polycyclic hydrocarbons (seen in *fungus in contaminated food), and HPV
Term
are there genetic risk factors for esophageal SCC?
Definition
yes, loss of tumor suppressor genes such as p53 and p16/INK4a can play a role
Term
where in the esophagus is SCC more common?
Definition
SCC is seen more commonly in the *upper/middle esophagus
Term
how does SCC usually start in the esophagus?
Definition
SCC usually starts as squamous dysplasia which appears as white patches. this can eventually grow into exophytic and polypoid masses that an encroach on the lumen of the esophagus, leading to increasing dysphagia (however, often progression is so slow making detection difficult)
Term
what local structures are often the first points of spread if esophageal SCC metastasizes? what potentiates this spread?
Definition
the respiratory tree, aorta, mediastinum and pericardium. b/c of insidious onset, most cases present w/advanced lesions which have invaded the esophageal wall. the esophagus itself has a rich lymphatic network which promotes the spread both longitudinally and circumferentially w/satellite lesions
Term
how differentiated are most cases of esophageal SCC?
Definition
moderately to well differentiated
Term
which lymph nodes are often infiltrated by upper esophageal SCC lesions?
Definition
the cervical lymph nodes
Term
which lymph nodes are often infiltrated by middle esophageal SCC lesions?
Definition
mediastinal, paratracheal, and tracheobronchial nodes
Term
what is a possible marker for esophageal cancer in terms of a pt's diet?
Definition
a slow change from solid to semisolid to liquid
Term
what are complications of esophageal SCC?
Definition
malnutrition, hemorrhage/sepsis, or aspiration via a tracheoesophageal fistula
Term
what is key but difficult to preventing death due to esophageal SCC?
Definition
early detection
Term
what is the overall 5 yr survival rate for pts with esophageal SCC?
Definition
9%
Term
where are esophageal adenocarcinomas usually found? how do they interact with the surrounding tissue? do they form glands?
Definition
the lower 1/3 of the esophagus and esophageal adenocarcinomas may invade the gastric cardia, infiltrate diffusely/invade deeply and usually will form glands/produce mucin
Term
what demographic are esophageal adenocarcinomas usually seen in?
Definition
white males in developed countries (*incidence is increasing)
Term
what is the relationship between barrett esophagus/GERD and esophageal adenocarcinomas?
Definition
there is a close association, barrett esophagus can progress to an esophageal adenocarcinoma via a series of genetic changes
Term
what are the genetic risk factors for an esophageal adenocarcinoma?
Definition
mutations of p53, amplification of c-ERB-B2, cyclin D1, cyclin E, mutation of retinoblastoma tumor suppressor gene, allelic loss of cyclin dependent kinase inhibitor p16/INK4a, and since TNF and NF kappa B dependent geners are overexpressed - inflammation likely plays a role
Term
what is the overall 5 yr survival rate for pts with esophageal adenocarcinoma?
Definition
25%
Term
what is the difference in ability to detect esophageal SCC vs an esophageal adenocarcinoma?
Definition
there are not usually many good markers for impending esophageal SCC but with esophageal adenocarcinomas, if a pt has barrett esophagus - then the dr should know to keep and eye out for adeno CAs in that area
Term
what are other less common kinds of CA that may be seen in the esophagus?
Definition
carcinoid tumors (chronic, button-like), melanoma (usually metastatic dark patches, but can be white if poorly differentiated), lymphoma, sarcoma, leiomyomas (benign smooth muscle CA), fibromyomas, and lipomas
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