GI Pathology - Congenital Abnormalities, Esophagus and Stomach Flashcards
What occurs in esophageal atresia?
Where does atresia occur most often? What is it often associated with it?
What is a less common type of atresia? What does it involve usually?
A thin, noncanalized cord replaces a segment of the esophagus, leading to obstruction.
At the tracheal bifurcation; associated with fistula that connects the upper and lower esophageal pouches to the trachea/bronchus.
Intestinal atresia which frequently involved the duodenum.
What is ectopia in terms of GI?
What are some examples?
Developmental rests
Ectopic gastric mucosa in the upper 1/3 of esophagus (inlet patch)
Ectopic pancreatic tissue occurs less often and is found in the esophagus and stomach
What causes an Omphalcele?
How is Gastroschisis different?
Incomplete closure of the abdominal musculature and the abdominal viscera herniates into the ventral membranous sac. They can be fixed surgically, but 40% of pts. have other congenital problems.
Gastroschisis includes all of the layers of the abdominal wall, from the peritoneum to the skin.
What is a Meckel diverticulum?
What causes it?
What is the “rule of 2”? (5)
A true diverticulum (blind outpouching that communicated with the parent lumen) in the ileum.
Failure of involution of the vitelline duct (connects developing gut to the yolk sac).
2% of the pop. 2 ft. from IC valve 2 in. long 2x more common in males Symptomatic by age 2 (but only 4% are symptomatic)
What sex is more likely to have pyloric stenosis? How common are they?
When does it present?
What is found on PE?
What is the treatment?
3-5x M>F; monozygotic twins; 1 in 300-900 live births.
3-6 wks into life with regurgitation, projectile vomiting after feeding, needs for re-feeding, etc.
Firm, ovoid mass (1-2 cm) in the abdomen.
Surgical splitting of the muscularis (myotomy) is curative.
What disease does Hirschsprung Dz have a 10% association with?
How common is it?
What is the pathogenesis?
What is a classic sign on XR?
What is the initial presentation?
Down syndrome
1/5000 live births
NCCs don’t migrate from the cecum to the rectum normally, leading to absence of the Meissner and Auerbach plexus and does not allow for coordinated contractions.
Megacolon.
Inability to pass meconium in the immediate neonatal period.
What is the blood supply to the regions of the esophagus?
How long is the esophagus?
Upper 1/3: inferior thyroid a.
Middle 1/3: branches of the thoracic aorta
Lower 1/3: left gastric a.
18-22 cm.
What are the 3 major causes of functional obstruction of the esophagus?
Nutcracker esophagus: high-amplitude contractions of the distal esophagus due to loss of normal coordination of the inner and outer layers of SM.
Diffuse esophageal spasm: repetitive, simultaneous contractions of the distal esophageal SM.
CREST syndrome
What are causes of mechanical obstruction of the esophagus? (3)
Stricture, stenosis, mass
- esophageal stenosis
- esophageal mucosal webs
- esophageal rings (Schatzki rings)
What is a Zenker diverticulum and when do they develop?
A diverticulum developed due to impaired relaxation of the cricopharyngeus muscle, which can be a trap for food and lead to bad halitosis. Most develop after age 50.
Achalasia is characterized by what triad?
What is primary vs. secondary?
Incomplete LES relaxation, increased LES tone, aperistalsis of the esophagus.
Primary is due to distal esophageal inhibitory neuronal (ganglion cell) degeneration. Secondary arises in Chagas disease, which causes destruction of the myenteric plexus, failure of peristalsis and esophageal dilation.
What GI bleed is more common: UGIB or LGIB?
UGIB is 4x more common
Reflux esophagitis is most commonly due to…
It is the…
Transient lower esophageal sphincter relaxation
Most common cause of esophagitis
Eosinophilic esophagitis symptoms…
Incidence is…
What is the usual cause?
Food impaction, dysphagia and feeding intolerance (infants).
Increasing significantly since 1978
Allergies to foods, asthma, rhinitis, atopic dermatitis, etc.
What is the pathogenesis of esophageal varices?
What is the major concern?
Portal HTN leads to development of collateral channel at sites where portal and caval systems communicate.
A variceal bleed, which is a medical emergency.
What is the only way to identify Barrett’s esophagus?
Endoscopy and biopsy, usually prompted by GERD
What 3 infections can pertain to the esophagus?
HSV
CMV
Candida albicans
What is Barrett’s esophagus characterized by?
What ages are most common?
What is the major risk?
Intestinal metaplasia within esophageal squamous mucosa
40-60 yo, more common in white males
Development of Adenocarcinoma
Which countries is Adenocarcinoma of the esophagus most common? (4)
Which group is at the highest risk?
What are risk factors?
What part of the esophagus is most common?
What histopathology is most common?
US, UK, Canada, Australia
7x more common in men
GERD, obesity, diet, etc.
Distal 1/3
Mucinous adenocarcinoma histopathology
Which countries is SCC of the esophagus most common? (3)
Who is at a greater risk?
50% occur where in the esophagus?
What are some risk factors? (8)
Iran, central China, Hong Kong
Adults > 45 yo; M>F 4:1; AA have 8x increased risk
Mid 1/3 of esophagus
Tobacco and alcohol Chemical/thermal injury (hot drinks) Esophageal disorders: achalasia/Plummeer-Vinson syndrome Radiation (5-10 yrs prior) Tylosis (RHBDF2 mutation) HPV +/- HIV
Normally, what are 2 damaging factors of the gastric mucosa?
What are protective factors? (5)
Gastric acidity, Peptic enzymes
Surface mucous secretion HCO3- secretion Mucosal blood flow Epithelial barrier function PGs
Mucosal gastric injury can be caused by what mediators? (6)
What can impair the submucosa? (3)
H pylori NSAID Tobacco EtOH Increased H+ Duodenal reflux
Ischemia, shock, NSAIDs
What is the difference of acute gastritis and gastropathy?
If neutrophils are present = acute gastritis
Few immune cells = gastropathy
What is the most common cause of chronic gastritis? Secondary most common?
H pylori is most common
10% are from autoimmune dz
Autoimmune gastritis is characterized by…
What is the clinical presentation linked to… (3)
What is the median age? Who is more likely to get it?
Diffuse mucosal damage to the oxyntic (acid-producting) mucosa within the body and fundus of the stomach.
Symptoms of anemia
Atrophic glossitis
Megaloblastosis of RBCs and epithelial cells
»B12 deficiency
Age 60; F>M
What are the causes of the following uncommon forms of gastritis?
Eosinophilic:
Lymphocytic (varioform gastritis):
Granulmatous:
Eosinophilic: allergies, immune disorders, parasites, H pylori
Lymphocytic (varioform gastritis): women, celiac disease
Granulmatous:Crohn disease*, Sarcoid and infection
Stress ulcers are most common in which patients?
What are Curling ulcers
What are Cushing ulcers?
Pts. w/ shock, sepsis or trauma.
Ulcers occuring in the proximal duodenum and associated with burns or trauma = Curling.
Gastric, duodenal and esophageal ulcers arising in pts. with intracranial disease = Cushing ulcers and carry a high incidence of perforation.
PUD =
Most common cause:
A chronic mucosal ulceration affecting the duodenum or stomach
NSAIDs, potentiated by corticosteroids
What are clinical symptoms of PUD?
Where is pain referred in penetrating ulcers?
Epigastric burning or aching pain 1-3 hrs. after meals/at night, or relieved with milk or OTC meds
Referred pain to the back, LUQ or chest
H. pylori infection presents as…
Virulence of the bug is linked to… (4)
Antral gastritis with normal or increased acid production
Flagella, urease, adhesins, toxin
What can be absorbed in the stomach? (3)
Aspirin
NSAIDs
EtOH
H. pylori (chronic gastritis)
Location Inflammatory infiltrate Acid production Gastrin Serology Sequelae
Location - antrum
Inflammatory infiltrate - neutrophils, subepithelium plasma cells
Acid production - increased to slightly decreased
Gastrin - normal to low
Serology - Abs to H. pylori
Sequelae - peptic ulcers, adenocarcinoma, MALToma
Autoimmune gastritis
Location Inflammatory infiltrate Acid production Gastrin Serology Sequelae
Location - body
Inflammatory infiltrate - lymphocytes, Mo
Acid production - decreased
Gastrin - increased
Serology - Abs to parietal cells
Sequelae - atrophy, anemia, adenocarcinoma
What are the 3 major causes of PUD?
What parts of the GI tract are mostly affected?
How does it affect secretion of gastric acid and duodenal bicarb?
H. pylori, NSAIDs, smoking
Gastric antrum or duodenum
Increased acid secretion and decreased bicarb secretion
PUD results from…
Imbalances between mucosal defense mechanisms and damaging factors that cause chronic gastritis
The classic peptic ulcer appears…
What appearance is more characteristic of cancer?
“Sharply punched-out defect”
Headed-up margins
What are the 2 hypertrophic gastropathies?
Menetrier disease and Zollinger-Ellison syndrome
What “characterizes” hypertrophic gastropathies?
Giant “cerebriform” enlargement of the rugal folds due to epithelial hyperplasia *without inflammation.
Zollinger-Ellison syndrome
Mean pt. age Location Predominant cell type Inflammatory infiltrate Symptoms Risk factors Associated with adenocarcinoma?
Mean pt. age - 50 yo Location - fundus Predominant cell type - parietal cells Inflammatory infiltrate - neutrophils Symptoms - peptic ulcers Risk factors - MEN Associated with adenocarcinoma? No
Menetrier disease
Mean pt. age Location Predominant cell type Inflammatory infiltrate Symptoms Risk factors Associated with adenocarcinoma?
Mean pt. age - 30-60 yo Location - body and fundus Predominant cell type - mucous cells Inflammatory infiltrate - limited Symptoms - hypoproteinemia, weight loss, diarrhea Risk factors - none Associated with adenocarcinoma? Yes
What are the 3 benign tumors of the stomach?
Inflammatory and hyperplastic polyps
Fundic gland polyps - associated with FAP
Gastric adenomas - association w/ adenocarcinoma
Most common benign tumor of the stomach?
Which one is associated with H. pylori? PPI? FAP?
What are the symptoms of each?
Which ones have malignant potential?
Inflammatory and hyperplastic polyps
H. pylori - Inflammatory and hyperplastic polyps
PPI - fundic gland polyps
FAP - fundic gland polyps
Similar to chronic gastritis. Fundic gland polyps may be ASX or have mild nausea.
Gastric adenomas are associated with adenocarcinoma. Fundic gland polyps have an association in the syndromic variants only (FAP).
Germ line loss of what tumor suppressor gene is associated with familial gastric carcinoma?
What “type” of gastric carcinoma?
CDH1, which encodes for E-cadherin, which aids in cell adhesion
Diffuse gastric cancer
Intestinal type gastric cancer is seen in which patients? (2)
What molecular changes occur? (3)
What is the patient age and sex?
Sporadic and FAP patients
Increased Wnt pathway signaling
Loss of function of APC
Gain of function of b-catenin
Mean age 55 yo, M>F
MALT lymphoma is associated with…
What is the most common translocation?
What lesion is diagnostic in the gastric glands?
H. pylori infection
t(11;18)(q21;q21)
Diagnostic lymphoepithelial lesions
Carcinoid tumor (NE carcinoma) of the jejunum and ileum
Fraction of GI carcinoid Mean patient age Location Size Secretory products Symptoms Behavior Disease associations
Fraction of GI carcinoid - >40% Mean patient age - 65 yo Location - throughout Size - <3.5 cm Secretory products - serotonin, substance P, polypeptide YY Symptoms - asymptomatic, incidental Behavior - aggressive Disease associations - none
GI carcinoid tumor appearance grossly:
High-mag:
EM:
Grossly: yellow/tan submucosal nodule
High-mag: salt and pepper pattern
EM: cytoplasmic dense core neurosecretory granules
What is the most common mesenchymal tumor of the abdomen?
What does it arise from?
GI Stromal Tumor
Interstitial cells of Cajal
What is the peak age for GIST?
How much of the stomach is impacted?
What is the prognosis related to?
75-80% of GIST have GOF in…
60 yo
Half of the stomach
Size, mitotic index and location
Tyrosine kinase KIT
