GI Pathology - Congenital Abnormalities, Esophagus and Stomach Flashcards

1
Q

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

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.

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2
Q

What is ectopia in terms of GI?

What are some examples?

A

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

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3
Q

What causes an Omphalcele?

How is Gastroschisis different?

A

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.

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4
Q

What is a Meckel diverticulum?

What causes it?

What is the “rule of 2”? (5)

A

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)
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5
Q

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?

A

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.

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6
Q

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?

A

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.

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7
Q

What is the blood supply to the regions of the esophagus?

How long is the esophagus?

A

Upper 1/3: inferior thyroid a.
Middle 1/3: branches of the thoracic aorta
Lower 1/3: left gastric a.

18-22 cm.

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8
Q

What are the 3 major causes of functional obstruction of the esophagus?

A

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

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9
Q

What are causes of mechanical obstruction of the esophagus? (3)

A

Stricture, stenosis, mass

  • esophageal stenosis
  • esophageal mucosal webs
  • esophageal rings (Schatzki rings)
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10
Q

What is a Zenker diverticulum and when do they develop?

A

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.

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11
Q

Achalasia is characterized by what triad?

What is primary vs. secondary?

A

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.

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12
Q

What GI bleed is more common: UGIB or LGIB?

A

UGIB is 4x more common

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13
Q

Reflux esophagitis is most commonly due to…

It is the…

A

Transient lower esophageal sphincter relaxation

Most common cause of esophagitis

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14
Q

Eosinophilic esophagitis symptoms…

Incidence is…

What is the usual cause?

A

Food impaction, dysphagia and feeding intolerance (infants).

Increasing significantly since 1978

Allergies to foods, asthma, rhinitis, atopic dermatitis, etc.

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15
Q

What is the pathogenesis of esophageal varices?

What is the major concern?

A

Portal HTN leads to development of collateral channel at sites where portal and caval systems communicate.

A variceal bleed, which is a medical emergency.

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16
Q

What is the only way to identify Barrett’s esophagus?

A

Endoscopy and biopsy, usually prompted by GERD

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17
Q

What 3 infections can pertain to the esophagus?

A

HSV
CMV
Candida albicans

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18
Q

What is Barrett’s esophagus characterized by?

What ages are most common?

What is the major risk?

A

Intestinal metaplasia within esophageal squamous mucosa

40-60 yo, more common in white males

Development of Adenocarcinoma

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19
Q

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?

A

US, UK, Canada, Australia

7x more common in men

GERD, obesity, diet, etc.

Distal 1/3

Mucinous adenocarcinoma histopathology

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20
Q

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)

A

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
21
Q

Normally, what are 2 damaging factors of the gastric mucosa?

What are protective factors? (5)

A

Gastric acidity, Peptic enzymes

Surface mucous secretion
HCO3- secretion
Mucosal blood flow
Epithelial barrier function
PGs
22
Q

Mucosal gastric injury can be caused by what mediators? (6)

What can impair the submucosa? (3)

A
H pylori
NSAID
Tobacco
EtOH
Increased H+
Duodenal reflux

Ischemia, shock, NSAIDs

23
Q

What is the difference of acute gastritis and gastropathy?

A

If neutrophils are present = acute gastritis

Few immune cells = gastropathy

24
Q

What is the most common cause of chronic gastritis? Secondary most common?

A

H pylori is most common

10% are from autoimmune dz

25
Q

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?

A

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

26
Q

What are the causes of the following uncommon forms of gastritis?

Eosinophilic:
Lymphocytic (varioform gastritis):
Granulmatous:

A

Eosinophilic: allergies, immune disorders, parasites, H pylori

Lymphocytic (varioform gastritis): women, celiac disease

Granulmatous:Crohn disease*, Sarcoid and infection

27
Q

Stress ulcers are most common in which patients?

What are Curling ulcers

What are Cushing ulcers?

A

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.

28
Q

PUD =

Most common cause:

A

A chronic mucosal ulceration affecting the duodenum or stomach

NSAIDs, potentiated by corticosteroids

29
Q

What are clinical symptoms of PUD?

Where is pain referred in penetrating ulcers?

A

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

30
Q

H. pylori infection presents as…

Virulence of the bug is linked to… (4)

A

Antral gastritis with normal or increased acid production

Flagella, urease, adhesins, toxin

31
Q

What can be absorbed in the stomach? (3)

A

Aspirin
NSAIDs
EtOH

32
Q

H. pylori (chronic gastritis)

Location
Inflammatory infiltrate
Acid production
Gastrin
Serology
Sequelae
A

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

33
Q

Autoimmune gastritis

Location
Inflammatory infiltrate
Acid production
Gastrin
Serology
Sequelae
A

Location - body
Inflammatory infiltrate - lymphocytes, Mo
Acid production - decreased
Gastrin - increased
Serology - Abs to parietal cells
Sequelae - atrophy, anemia, adenocarcinoma

34
Q

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?

A

H. pylori, NSAIDs, smoking

Gastric antrum or duodenum

Increased acid secretion and decreased bicarb secretion

35
Q

PUD results from…

A

Imbalances between mucosal defense mechanisms and damaging factors that cause chronic gastritis

36
Q

The classic peptic ulcer appears…

What appearance is more characteristic of cancer?

A

“Sharply punched-out defect”

Headed-up margins

37
Q

What are the 2 hypertrophic gastropathies?

A

Menetrier disease and Zollinger-Ellison syndrome

38
Q

What “characterizes” hypertrophic gastropathies?

A

Giant “cerebriform” enlargement of the rugal folds due to epithelial hyperplasia *without inflammation.

39
Q

Zollinger-Ellison syndrome

Mean pt. age
Location
Predominant cell type
Inflammatory infiltrate
Symptoms
Risk factors
Associated with adenocarcinoma?
A
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
40
Q

Menetrier disease

Mean pt. age
Location
Predominant cell type
Inflammatory infiltrate
Symptoms
Risk factors
Associated with adenocarcinoma?
A
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
41
Q

What are the 3 benign tumors of the stomach?

A

Inflammatory and hyperplastic polyps

Fundic gland polyps - associated with FAP

Gastric adenomas - association w/ adenocarcinoma

42
Q

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?

A

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).

43
Q

Germ line loss of what tumor suppressor gene is associated with familial gastric carcinoma?

What “type” of gastric carcinoma?

A

CDH1, which encodes for E-cadherin, which aids in cell adhesion

Diffuse gastric cancer

44
Q

Intestinal type gastric cancer is seen in which patients? (2)

What molecular changes occur? (3)

What is the patient age and sex?

A

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

45
Q

MALT lymphoma is associated with…

What is the most common translocation?

What lesion is diagnostic in the gastric glands?

A

H. pylori infection

t(11;18)(q21;q21)

Diagnostic lymphoepithelial lesions

46
Q

Carcinoid tumor (NE carcinoma) of the jejunum and ileum

Fraction of GI carcinoid
Mean patient age
Location
Size
Secretory products
Symptoms
Behavior
Disease associations
A
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
47
Q

GI carcinoid tumor appearance grossly:

High-mag:

EM:

A

Grossly: yellow/tan submucosal nodule

High-mag: salt and pepper pattern

EM: cytoplasmic dense core neurosecretory granules

48
Q

What is the most common mesenchymal tumor of the abdomen?

What does it arise from?

A

GI Stromal Tumor

Interstitial cells of Cajal

49
Q

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…

A

60 yo

Half of the stomach

Size, mitotic index and location

Tyrosine kinase KIT