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
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
26
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
27
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.
28
PUD = Most common cause:
A chronic mucosal ulceration affecting the duodenum or stomach NSAIDs, potentiated by corticosteroids
29
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
30
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
31
What can be absorbed in the stomach? (3)
Aspirin NSAIDs EtOH
32
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
33
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
34
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
35
PUD results from...
Imbalances between mucosal defense mechanisms and damaging factors that cause chronic gastritis
36
The classic peptic ulcer appears... What appearance is more characteristic of cancer?
"Sharply punched-out defect" Headed-up margins
37
What are the 2 hypertrophic gastropathies?
Menetrier disease and Zollinger-Ellison syndrome
38
What "characterizes" hypertrophic gastropathies?
Giant "cerebriform" enlargement of the rugal folds due to epithelial hyperplasia *without inflammation.
39
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 ```
40
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 ```
41
What are the 3 benign tumors of the stomach?
Inflammatory and hyperplastic polyps Fundic gland polyps - associated with FAP Gastric adenomas - association w/ adenocarcinoma
42
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).
43
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
44
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
45
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
46
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 ```
47
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
48
What is the most common mesenchymal tumor of the abdomen? What does it arise from?
GI Stromal Tumor Interstitial cells of Cajal
49
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