Robbins GI morphologies

Question 1

Congenital malformations of the GI tract: key concepts and disorders

Answer 1

1. The GI tract is a common site of developmental abnormalities: one abnormality should compel us to look for other organ anomalies.
2. Atrias, fistulas, imperforate anus, Meckel’s Diverticulum, Zenkers Diverticulum, Congenital Hypertrophic Stenosis, Pyloric Stenosis, Hirschsprungs Dx, Ectopia, Diphragmatic herniatinon
3. Atresia, and fistulas: structural developmental anomalies that disrupt normal gastrointestinal transit. typically present early in life. Imperforate anus is the most common form of congenital intestinal atresia, while the esophagus is the most common site of fistulization.
4. Stenosis may be developmental or acquired. Both forms are characterized by a thickened wall and partial or complete luminal obstruction. Acquired forms are often due to inflammatory scarring.
5. Diaphragmatic hernia is characterized by incomplete diaphragm development and herniation of abdominal organs into the thorax. This often results in pulmonary hypoplasia. Omphalocele andgastroschisis refer to ventral herniation of abdominal organs.
6. Ectopia refers to the presence of normally formed tissues in an abnormal site. This is common in the gastrointestinal tract, with ectopic gastric mucosa in the upper third of the esophagus being the most common form.
7. The Meckel diverticulum is a true diverticulum, defined by the presence of all three layers of the bowel wall, that reflects failed involution of the vitelline duct. It is common and is a frequent site of gastric ectopia, which may result in occult bleeding.
8. Congenital hypertrophic pyloric stenosis is a form of obstruction that presents between the third and sixth weeks of life. There is an ill-defined genetic component to this disease, which is most common in males.
9. Hirschsprung disease is caused by the absence of neural crest derived ganglion cells within the colon. It causes functional obstruction of the affected bowel and proximal dilation. The defect always begins at the rectum, but extends proximally for variable

Question 2

Esophageal Diseases: key concepts and ideas

Answer 2

1. Esophageal Diseases: achalasia, mallory weis syn, esophagitis, barrots, adenocarcinoma, squamous cell, varices
2. Abnormalities of esophageal motility: nutcracker esophagus, diffuse esophageal spasm
3. Achalasia: primary or secondary, latter form most commonly due to Trypanosoma cruzi infection.
4. Mallory-Weiss tears of mucosa at gastroesophageal junction develop as a result of severe retching or vomiting.
5. Esophagitis:
   
   1. chemical or infectious mucosal injury.
   2. Infection most common in immunocompromised individuals
   3. Most prevalent cause: gastroesophageal reflux disease (GERD).
   4. Eosinophilic esophagitis associated with food allergy, allergic rhinitis, asthma, or modest peripheral eosinophilia.
   5. common cause of GERD-like symptoms in children living in developed countries.
6. Gastroesophageal varices consequence of portal hypertension
   
   1. present in nearly half of cirrhosis patients.
7. Barrett esophagus
   
   1. develops from chronic GERD
   2. represents columnar metaplasia, esophageal squamous mucosa.
   3. risk factor for development of esophageal adenocarcinoma .
8. Esophageal squamous cell carcinoma:
   
   1. associated with alcohol, tobacco use, poverty, caustic esophageal injury, achalasia, tylosis, and Plummer-Vinson syndrome.

Question 3

hematoxylin and eosin–stained sections show no signs of acetylcholinesterase. preoperative barium enema study showing constricted rectum. dilated sigmoid colon

Answer 3

Hirschsprung disease.

Question 4

neutrophils present as well as necrosis

Answer 4

The morphology of chemical and infectious esophagitis varies with etiology. Dense infiltrates of neutrophils are present in most cases but may be absent following injury induced by chemicals (lye, acids, or detergent), which can lead to outright necrosis of the esophageal wall.

Question 5

ulceration, superficial necrosis, granulation tissue and eventual fibrosis.

Answer 5

Pill-induced esophagitis frequently occurs at the site of strictures that impede passage of luminal contents. When present, ulceration is accompanied by superficial necrosis with granulation tissue and eventual fibrosis.

Question 6

neutrophils, intimal proliferation, luminal narrowing of submucosal and mural blood vessels. mucosal damage

I

Answer 6

Esophageal irradiation causes damage similar to that seen in other tissues and includes intimal proliferation and luminal narrowing of submucosal and mural blood vessels. The mucosal damage is, in part, secondary to this radiation-induced vascular injury as discussed in Chapter 9 .

I

Question 7

adherent, gray-white pseudomembranes composed of densely matted fungal hyphae and inflammatory cells covering the esophageal mucosa.

Answer 7

1. Infection by fungi or bacteria can either cause injury or complicate a preexisting ulcer. Nonpathogenic oral bacteria are frequently found in ulcer beds, while pathogenic organisms, which account for about 10% of infectious esophagitis, may invade the lamina propria and cause necrosis of overlying mucosa. Candidiasis, in its most advanced form, is characterized by adherent, gray-white pseudomembranes composed of densely matted fungal hyphae and inflammatory cells covering the esophageal mucosa.
   2.

Question 8

punched-out ulcer, nuclear viral inclusions, rim of degenerating epithelial cells at the margin of the ulcer

Answer 8

The endoscopic appearance often provides a clue as to the infectious agent in viral esophagitis. Herpes viruses typically cause punched-out ulcers ( Fig. 17-4 A ). Biopsy specimens demonstrate nuclear viral inclusions within a rim of degenerating epithelial cells at the margin of the ulcer ( Fig. 17-4 B ).

Question 9

shallow ulcerations, nuclear and cytoplasmic inclusions in capillary endothelium and stromal cells.

Answer 9

CMV causes shallower ulcerations and characteristic nuclear and cytoplasmic inclusions within capillary endothelium and stromal cells ( Fig. 17-4 C )

Question 10

dysphagia for solids and liquids, difficulty in belching, chest pain. incomplete LES relaxation, increased LES tone, and aperistalsis of the esophagus

Answer 10

achalasia

Question 11

distal esophageal inhibitory ganglion cell degeneration

Answer 11

Primary achalasia

Question 12

Secondary achalasia

Answer 12

possibly caused by Chagas disease: Trypanosoma cruzi infection causes destruction of the myenteric plexus, failure of peristalsis, and esophageal dilatation. Duodenal, colonic, and ureteric myenteric plexuses can also be affected in Chagas disease.

Question 13

Achalasia like disease

Answer 13

1. may be caused by
   
   1. diabetic autonomic neuropathy
   2. infiltrative disorders: malignancy, amyloidosis, or sarcoidosis
   3. lesions of dorsal motor nuclei, particularly polio or surgical ablation
   4. association with
      
      1. Down syndrome
      2. Allgrove (triple A) syndrome
   5. remote herpes simplex virus 1 (HSV1) infection
   6. immunoregulatory gene polymorphisms to
   7. Sjögren syndrome
   8. autoimmune thyroid disease

Question 14

basal epithelial cell apoptosis, mucosal atrophy, and submucosal fibrosis without significant acute inflammatory infiltrates.

Answer 14

esophageal graft-versus-host disease

Question 15

Heterozygous loss-of-function mutations in the receptor tyrosine kinase RET

Answer 15

Hirschsprung disease

Question 16

Nutcracker esophagus

Answer 16

Nutcracker esophagus describes patients with high-amplitude contractions of the distal esophagus that are, in part, due to loss of the normal coordination of inner circular layer and outer longitudinal layer smooth muscle contractions.

Question 17

repetitive, simultaneous contractions of the distal esophageal smooth muscle.

Answer 17

Diffuse esophageal spasm is characterized by repetitive, simultaneous contractions of the distal esophageal smooth muscle.

Question 18

1. high resting pressure or incomplete relaxation
2. high resting pressure or incomplete relaxation+absence altered patterns of esophageal contractio

Answer 18

1. Lower esophageal sphincter dysfunction, such as high resting pressure or incomplete relaxation, are present in many patients with nutcracker esophagus or diffuse esophageal spasm.
2. In the absence altered patterns of esophageal contraction, these sphincter abnormalities are termed hypertensive lower esophageal sphincter .

Question 19

impaired relaxation and spasm of the cricopharyngeus muscle after swallowing

Answer 19

may result in increased pressure within the distal pharynx and development of a Zenker diverticulum (pharyngoesophageal diverticulum), which is located immediately above the upper esophageal sphincter. Zenker diverticulae are uncommon, but typically develop after age 50

Question 20

fibrous thickening of the submucosa and is associated with atrophy of the muscularis propria as well as secondary epithelial damage.

Answer 20

Benign esophageal stenosis, or narrowing of the lumen

Question 21

functional obstruction vs malignant strictures

Answer 21

functional obstruction or benign strictures maintain their appetite and weight, while, as discussed later, malignant strictures are often associated with weight loss.

Question 22

ledge-like protrusions of mucosa: which conditions are they associated with?

Answer 22

Esophageal mucosal webs:

1. gastroesophageal reflux
2. chronic graft-versus-host disease
3. blistering skin diseases

Question 23

beefy red tongue, and fissuring and dry scaling of lips, dysphagia, weakness, paleness

Answer 23

Paterson-Brown-Kelly /Plummer-Vinson syndrome syndrome

difficulty in swallowing, usually accompanied by generalized weakness due to anemia, and dryness of the mouth with burning of the tongue

glossitis, hypochromic (iron deficient) anemia, and esophageal webs

Question 24

nonprogressive dysphagia associated with incompletely chewed food.

Answer 24

the main symptom of esophageal webs

Question 25

thickened mucosa, submucosa, distal esophagus, above gastroesophageal junction, covered by squamous cells

Answer 25

Schatzki rings, kind of like esophageal webs

thickened mucosa, submucosa, and possibly hypertrophic muscularis propria.

above gastroesophageal sphincter = A signet rings

Question 26

thick gastric cardia-type mucosa on a ledge like protrusion located at the squamocolumnar junction

Answer 26

Schatzki rings, B rings

Question 27

1. eosinophils, neutrophils in the squamous mucosa followed by neutrophils
2. Basal zone hyperplasia exceeds 20% of the total epithelial thickness and elongation of lamina propria papillae, extend into the upper third of the epithelium

Answer 27

1. Eosinophilic esophagitis is characterized by numerous intraepithelial eosinophils. Abnormal squamous maturation is also apparent.
2. Reflux esophagitis with scattered intraepithelial eosinophils and mild basal zone expansion.

Question 28

patches of red, velvety mucosa extending upward from the gastroesophageal junction. metaplastic mucosa alternates with residual smooth, pale squamous mucosa and interfaces with light-brown columnar (gastric) mucosa distally

Answer 28

Barretts

Question 29

intestinal-type metaplasia with goblet cells. above the gastroesophageal junction. mucous vacuoles stain pale blue.

Answer 29

Diagnosis of Barrett esophagus requires endoscopic evidence of metaplastic columnar mucosa above the gastroesophageal junction.

Question 30

1. Atypical mitoses, nuclear hyperchromasia, irregularly clumped chromatin, increased nuclear-to-cytoplasmic ratio. immature cells in full thickness. Gland budding.etaplastic columnar mucosa above the gastroesophageal junction. intestinal-type metaplasia
2. what is this called and how is it classified?

Answer 30

1. Barrett esophagus with dysplasia: metaplastic columnar mucosa above the gastroesophageal junction. Intestinal-type metaplasia is seen as replacement of the squamous esophageal epithelium with goblet cells.
2. classified as low grade or high grade. Atypical mitoses, nuclear hyperchromasia, irregularly clumped chromatin, increased nuclear-to-cytoplasmic ratio, and a failure of epithelial cells to mature as they migrate to the esophageal surface are present in both grades of dysplasia (Fig. 17-8 A ). Gland architecture is frequently abnormal and is characterized by budding, irregular shapes, and cellular crowding.

Question 31

f metaplastic columnar mucosa above the gastroesophageal junction. intestinal-type metaplasia, distinct mucous vacuoles, epithelial cells invade the lamina propria

Answer 31

Barrett esophagus: High-grade dysplasia

Question 32

distal third of the esophagus, flat to raised patches producing mucin, and intact mucosa, masses 5 cm or more

Answer 32

1. Esophageal adenocarcinoma usually occurs in the distal third of the esophagus and may invade the adjacent gastric cardia
2. Initially appearing as flat or raised patches in otherwise intact mucosa, large masses of 5 cm or more in diameter may develop.

Question 33

Barrett esophagus present adjacent to the tumor, which produces mucin and forms glands, intestinal-type morphology

Answer 33

Esophageal adenocarcinoma

tumors may infiltrate diffusely or ulcerate and invade deeply. Microscopically, Barrett esophagus is frequently present adjacent to the tumor. Tumors most commonly produce mucin and form glands ( Fig. 17-10 A ), often with intestinal-type morphology; less frequently tumors are composed of diffusely infiltrative signet-ring cells (similar to those seen in diffuse gastric cancers) or, in rare cases, small poorly differentiated cells (similar to small-cell carcinoma of the lung).

Question 34

mutation of TP53, low activity of CDKN2A, also known asp16/INK4a,

Answer 34

1. progression of Barrett esophagus to adenocarcinoma: early stage
2. identified in nondysplastic Barrett metaplasia persist and accumulate mutations during progression to dysplasia and invasive carcinoma.
3. Chromosomal abnormalities, mutation of TP53 , and downregulation of the cyclin-dependent kinase inhibitor CDKN2A, also known asp16/INK4a, are detected at early stages.

Question 35

amplification of EGFR, ERBB2, MET, cyclin D1 , and cyclin E genes.

Answer 35

1. progression of Barrett esophagus to adenocarcinoma: late stage
2. occurs over an extended period through the stepwise acquisition of genetic and epigenetic changes.
3. Later during progression there is amplification of EGFR, ERBB2, MET, cyclin D1 , and cyclin E genes.

Question 36

middle third of the esophagus . small, gray-white, plaque-like thickenings.

Answer 36

1. squamous dysplasia
2. In contrast to adenocarcinoma, half of squamous cell carcinomas occur in the middle third of the esophagus
3. Squamous cell carcinoma begins as an in situ lesion termed squamous dysplasia (this lesion is referred to as intraepithelial neoplasia or carcinoma in situ at other sites).
4. Early lesions appear as small, gray-white, plaque-like thickenings.

Question 37

1. Most squamous cell carcinomas are…
2. symptomatic tumors are generally….
3. sites of lymph node metastases….

Answer 37

1. Most squamous cell carcinomas are moderately to well-differentiated
2. symptomatic tumors are generally very large at diagnosis and have already invaded the esophageal wall.
3. The rich lymphatic network promotes circumferential and longitudinal spread. The sites of lymph node metastases vary with tumor location:
4. cancers in the upper third of the esophagus favor cervical lymph nodes; those in the middle third favor mediastinal, paratracheal, and tracheobronchial nodes; and those in the lower third spread to gastric and celiac nodes.

Question 38

cancers in the:

1. upper third of the esophagus favor
2. middle third favor
3. lower third spread to gastric and celiac nodes.

Answer 38

1. squamous cell carcinomas:
   
   1. upper third of the esophagus favor cervical lymph node
   2. middle third favor mediastinal, paratracheal, and tracheobronchial nodes
   3. lower third spread to gastric and celiac nodes.