Download Esophagus Pathology ©2010 Mark Tuttle Achalasia ↓ LES

Esophagus Pathology
©2010 Mark Tuttle
Achalasia
↓ LES relaxation, ↑LES tone, aperistalsis of the esophagus
Atresia
Fistula
Barrett esophagus
Thin, noncanalized cord replaces a segment of esophagus
Abnormal cnxn of two epithelium-lined organs or vessels
Intestinal metaplasia within the esophageal squamous
mucosa. Secondary to GERD
Blind outpouching of the GI that is lined by mucosa,
communicates with the lumen, and includes all 3 layers of
the bowel wall (Lamina propria, submucosa, muscularis
externa)
Diverticulum above the UES (upper esophageal sphincter)
Diverticulum
Zenker’s diverticulum
Mallory-Weiss
Syndrome
Melena
Hematemesis
Mucosal webs
Plummer-Vinson
Syndrome
Rings
Longitudinal lacerations near gastroesophageal junction.
Often from severe vomiting.
Black tar stools  upper GI bleed
Throwing up blood
Obstructive wedge-like fibrovascular connective tissue
and overlying epithelium
Esophageal webs + iron-deficiency anemia, glossitis, and
cheilosis
Like webs, but circumferential.
A webs: upper (squamous), B webs: lower esophagus
Traction diverticulum
Diverticulum in the mid esophagus
Pyrosis
Heartburn
Epiphrenic diverticulum
Diverticulum just above LES (lower esophageal sphincter)
Varices
Distention of esophageal veins in portal circulation
Hiatal hernia
Herniation of visceral material through diaphragm hiatus
Dyspepsia
Indigestion, pain in upper abdomen
Disease
Signs & Symptoms
Pathogenesis
Histology
Atresia + fistula
- aspiration,
- Tracheoesophageal fistulas are more common.
- suffocation
- Occurs most often at tracheal bifurcation and
- pneumonia
frequently associated with atresia.
- fluid and electrolyte imbalances
- Associated anomalies are congenital heart disease
Most common
 Must be corrected early b/c incompatible with life.
and neurologic, GU, and other GI anomalies.
Stenosis
- GERD, radiation, scleroderma, caustic injury
- Fibrous thickening of walls
- Progressive dysphagia; if severe, obstruction
- Usually due to severe injury and resultant scarring
Mucosal Webs
- Upper esophagus
- Ledge-like protrusions from into the lumen
- Semicircumferential
- Most often encountered in women >40 years old and are of uncertain etiology
- Episodic dysphagia when swallowing large amounts of food.
Mucosal Rings
- Lower esophagus
- Concentric
Plummer-Vinson - Combination of 1) upper webs, 2) iron deficiency
Syndrome
anemia with 3) glossitis and 4)cheilosis
- Risk of post-cricoid carcinoma
Achalasia
1) ↓ LES relaxation
- Primary (idiopathic) (most common)
2) ↑LES tone
o dysfunction of inhibitory neurons in the distal
3) Aperistalsis of the esophagus
esophagus
- Manifests in young adulthood
o neuronal degeneration of intrinsic or extrinsic
- Leads to progressive dysphagia, regurgitation, and
(vagal) nerves
aspiration
- Secondary
- Aspiration pneumonia, airway obstruction, candidiasis,
o Chagas disease (Trypanosoma cruzi)
- Dilated esophagus above LES
lower esophageal diverticula and 5% squamous cell
o lesions of vagal motor nuclei (i.e. polio)
- Diminished myenteric ganglia in
carcinoma of the esophagus
o diabetic autonomic neuropathy
body of esophagus and possibly
o Infiltrative disorders (malignancy, sarcoidosis, etc.) in the region of the LES
Disease
Hiatal Hernia
- Cause unknown
- Can cause GERD
Diverticulae
Zenker
(Pharyngeoesophageal)
Signs & Symptoms
- Hernia of part of stomach thru esophageal foramen
- Separation/widening of diaphragmatic crura
- 1-20% of adult population affected
- Incidence increases with age, but also seen in kids
- Complications
o Ulcerations, bleeding and perforations
o Strangulation or obstruction (paraesophageal hernias)
o Reflux esophagitis
Traction
- accumulates food, causing :
o regurgitation
o dysphagia
o a neck mass
o aspiration pneumonia
- asymptomatic
Epiphrenic
- nocturnal regurgitation of massive amounts of fluid
Lacerations
(Mallory-Weiss
Syndrome)
- Most commonly seen in alcoholics and associated with
severe retching or vomiting
- Underlying hiatal hernia is a known risk factor
- Accounts for 5-10% of the upper GI bleeding episodes
- Bleeding usually ceases without surgical intervention,
but massive hematemesis may occur
- Infection of mucosal defect can lead to inflammatory
ulcer or mediastinitis
Circulatory Disorders
Esophageal Varices  Portal
hypertension
- dilated, tortuous
vessels
- ↑pressure in
plexus gives rise
to varices
-
Usually asymptomatic until rupture
With rupture, there is life-threatening, massive
hematemesis (but varices only cause <50% of all
hematemesis episodes)
Half the deaths of patients with advanced cirrhosis of the
liver result from a ruptured varix.
Death from hemorrhage or triggered hepatic coma
Treatment usually requires endoscopic injection of a
thrombotic agent (sclerotherapy) or balloon tamponade
40-50% of patients die in the first bleeding episode;
similar mortality rate in repeat episodes
Pathogenesis
Histology
- Sliding (axial): 95% of cases
o cardia of stomach herniates upward, as if it slid straight up
o Bell-shaped dilation of the stomach above the diaphragm
bounded by diaphragm
- Paraesophageal (rolling or nonaxial):
o separate portion of the stomach (usually greater curvature)
herniates alongside of the esophagus
o Associated w/ previous surgery (operations for sliding
hernias)
- located immediately above the UES
- caused by cricopharyngeal motor dysfunction w/ or
w/out GERD and diminished luminal size of the UES
- located at the midpoint of the esophagus
- caused by scarring from mediastinal
lymphadenitis(?), motor dysfunction, or congenital
- located above LES
- associated with discoordinated peristalsis and LES
relaxation
- Longitudinal tears at the esophagogastric junction Found astride the
or gastric cardia
esophagogastric junction or in
- Gastric musculature fails to relax properly, leading to the proximal gastric mucosa
massive dilation and tearing of the wall
- May involve only mucosa, but can penetrate and
cause rupture (Boerhaave syndrome)
- Most often associated with ALCOHOLIC
CIRRHOSIS and develop in 90% of cirrhotic
patients
- Collateral bypass channels develop where
portal and caval systems are in
communication.
1) Portal vein hypertension
2) Gastro-esophageal anastamoses
a. Coronary Veins in Stomach
b. Submucosal Plexus In Distal
Esophagus Rupture if >5mm
3) Azygous vein
4) Systemic circulation
Varix
Inflammatory Disorders
Disease
Signs & Symptoms
Esophagitis
- Inflammation of the esophageal mucosa
- 5% of adult population in US and Western
countries
Gastroesophageal
- Reflux of gastric into the lower esoph.
Reflux Disease
- Most important cause of esophagitis
(GERD)
- Severity of the symptoms does NOT
correlate to degree of histologic change
- Usually limited to - Symptoms
adults >40 yrs
o Heartburn
o Regurgitation of a sour brash
o Dysphagia
o Hematemesis, Melena (if severe)
- Complications
o Ulceration, bleeding, healing
o Barrett esophagus
o Stricture
Barrett Esophagus
- Metaplastic change whereby squamous
epithelium  columnar epithelium due to
- Common
prolonged injury
complication of
- Alteration in the differentiation program of
long-standing
esophageal mucosal stem cells
GERD (up to 10%)
- 40-60 yr old,
- Single most important risk factor for
white, M>F
esophageal adenocarcinoma
- Secondary
o 30-40x with > 3cm Barrett change
complications of
local ulceration
- Two requirements for diagnosis
with bleeding and
1. Endoscopy: columnar epi ↑the GE jxn
stricture
2. Histologic biopsy: intestinal metaplasia
Infectious &
Chemical
Esophagitis
- Infectious
o Viral: HSV and CMV in immunosuppressed
o Fungal (Immunosuppressed or Diabetics)
 Candidiasis (most common)
 Mucormycosis and Aspergillosis (rare)
- Mucosal irritants
o Corrosives & alkalis, cytotoxic drugs,
alcohol, hot fluids, heavy smoking
- Uremia
Pathogenesis
- Caused by a variety of physical, chemical, and
biologic agents
Histology
- Cause: mostly action of gastric juices on
esophagus
o Sliding hiatal hernia
o Decreased LES tone
 Alcohol, chocolate, cigarette smoking,
estrogen
 CNS Depressants e.g. morphine, valium
 Nasogastric intubation
o Inadequate or slowed esophageal clearance
o Delayed gastric emptying & ↑ gastric volume
o Reduced reparative capacity of esophageal
mucosa by extended exposure to gastric juices
- Sheets, patches, or a band of red velvety epithelium
(gross and endoscopy).
- Squamous epithelium replaced by columnar, with either
gastric surface and glandular mucus-secreting cells or
mixture of gastric and intestinal epithelium.
- Look for dysplasia as precursor to malignancy
HSV: “Punched out” ulcers
HSV: Nuclear inclusions
1. Inflammatory cells in
epithelial layer e.g.
neutrophils (indicates
severity), eosinophils
(early), and lymphocytes
2. Basal zone hyperplasia
in excess of 20% of
epithelial thickness.
3. Elongation of lamina
propria and congestion.
Neoplasms
Disease
Squamous Cell
Carcinoma
- 90% of
esophageal
cancers
worldwide; but
equal incidence
to adenocarcioma
in the US
- Adults over 50 yrs
- M:F; 2:1-20:1; 4:1
in US
- Incidence varies
between regions
- Blacks throughout
the world have a
higher risk, 4X in
US
Adenocarcinoma
- Incidence equal
to squamous cell
carcinoma in the
US
- >40 with median
age 60
- M>W; whites >
black.
Signs & Symptoms
- Clinical Features
o Insidious onset with late development of
symptoms
o Dysphagia, obstruction, cachexia,
debilitation
o Patients often adjust their diets and fail to
report symptoms until more severe
- Complications
o Hemorrhage and sepsis from ulceration
o Aspiration from tracheoesophageal fistula
- Prognosis
o 5-year survival 9%; 75% for superficial
lesions; 25% for surgical resection of
advanced lesions
o Recurrence local and at distant sites
o Lymph node metastasis reduces survival
rate
o Screening programs are less successful in
USA so prognosis is worse
Pathogenesis
Etiology and Pathogenesis
- Dietary, environmental, and genetic
- US - Alcohol and tobacco
- Other countries - fungi and nitrosamines
o Less use of these preservatives in US
- Chronic esophagitis  dysplasia  cancer
Histology
- Protruded (exophytic) (60%)
- Flat (15%): infiltrative
o Spreads in wall, thickens wall and arrows lumen
- Excavated (25%): necrotic ulceration
o deep infiltration sometimes fistulates into trachea (leading
to pneumonia) or into aorta (leading to hemorrhage into
the mediastinum and pericardium)
- Begins in situ
- 20% upper third, 50% middle third and 30% lower third
- Quite large by time symptoms elaborated
- Local circumferential and longitudinal spread by lymphatics
- Metastasizes to regional nodes
- Dysphagia predominant sign with weight
loss, hemorrhage, chest pain and vomiting
also noted.
- Prognosis - 5 year
- <20% overall
- >80% with resected superficial tumors
- Virtually all arise in Barrett esophagus
- 10% lifetime risk in Barret esophagus
- Documented genetic alterations
o p53 protein over expression in Barrett esophagus
o p53 functional inactivation due to point mutations leading
to loss of cell cycle control
Histology
1) Distal esophagus and may invade cardia
2) Start as flat or raised patches that become either large
nodules or infiltrate and ulcerate.
3) Most are mucin producers demonstrating intestinal-type
epithelium although some gastric-type is observed.
Histology