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2H SystemicPatho GIT > Chapter 17: GIT- Hypertrophic Gastropathies > Flashcards

Chapter 17: GIT- Hypertrophic Gastropathies Flashcards

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1
Q

What are Hypertrophic gastropathies?

A

are uncommon diseases characterized by giant cerebriform enlargement of the rugal folds due to epithelial hyperplasia without inflammation.

As might be
expected, the hypertrophic gastropathies are linked to excessive growth factor release.

The two
most well-understood examples are Ménétrier disease and Zollinger-Ellison syndrome, the
morphologic features of which are compared with other gastric proliferations in Table 17-4 .

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2
Q

TABLE 17-4 – Hypertrophic Gastropathies and Gastric Polyps

A
  • Ménétrier Disease (adult)
  • Zollinger- Ellison Syndrome
  • Inflammatory and Hyperplastic Polyps
  • Gastritis Cystica
  • Fundic Gland Polyps
  • Gastric Adenomas
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3
Q

TABLE 17-4 – Hypertrophic Gastropathies and Gastric Polyps

Mean patient
age (yr)

A
  • Ménétrier Disease (adult)
    • 30–60
  • Zollinger- Ellison Syndrome
    • 50
  • Inflammatory and Hyperplastic Polyps
    • 50–60
  • Gastritis Cystica
    • Variable
  • Fundic Gland Polyps
    • 50
  • Gastric Adenomas
    • 50–60
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4
Q

TABLE 17-4 – Hypertrophic Gastropathies and Gastric Polyps

Location

A
  • Ménétrier Disease (adult)
    • Body and fundus
  • Zollinger- Ellison Syndrome
    • Fundus
  • Inflammatory and Hyperplastic Polyps
    • Antrum > body
  • Gastritis Cystica
    • Body
  • Fundic Gland Polyps
    • Body and fundus
  • Gastric Adenomas
    • Antrum > body
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5
Q

TABLE 17-4 – Hypertrophic Gastropathies and Gastric Polyps

Predominant
cell type

A
  • Ménétrier Disease (adult)
    • Mucous
  • Zollinger- Ellison Syndrome
    • Parietal > mucous, endocrine
  • Inflammatory and Hyperplastic Polyps
    • Mucous
  • Gastritis Cystica
    • Mucous, cyst-lining
  • Fundic Gland Polyps
    • Parietal and chief
  • Gastric Adenomas
    • Dysplastic, intestinal
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6
Q

TABLE 17-4 – Hypertrophic Gastropathies and Gastric Polyps

Inflammatory
infiltrate

A
  • Ménétrier Disease (adult)
    • Limited, lymphocytes
  • Zollinger- Ellison Syndrome
    • Neutrophils
  • Inflammatory and Hyperplastic Polyps
    • Neutrophils and lymphocytes
  • Gastritis Cystica
    • Neutrophils and lymphocytes
  • Fundic Gland Polyps
    • None
  • Gastric Adenomas
    • Variable
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7
Q

TABLE 17-4 – Hypertrophic Gastropathies and Gastric Polyps

Symptoms

A
  • Ménétrier Disease (adult)
    • Hypoproteinemia, weight loss, diarrhea
  • Zollinger- Ellison Syndrome
    • Peptic ulcers
  • Inflammatory and Hyperplastic Polyps
    • Chronic gastritis
  • Gastritis Cystica
    • Chronic gastritis
  • Fundic Gland Polyps
    • None, nausea
  • Gastric Adenomas
    • Chronic gastritis
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8
Q

TABLE 17-4 – Hypertrophic Gastropathies and Gastric Polyps

Risk factors

A
  • Ménétrier Disease (adult)
    • None
  • Zollinger- Ellison Syndrome
    • Multiple endocrine neoplasia
  • Inflammatory and Hyperplastic Polyps
    • Chronic gastritis, H. pylori
  • Gastritis Cystica
    • Trauma, prior surgery
  • Fundic Gland Polyps
    • PPIs, FAP
  • Gastric Adenomas
    • Chronic gastritis, atrophy, intestinal metaplasia
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9
Q

What is Ménétrier disease?

A

is a rare disorder caused by excessive secretion of transforming growth factor α (TGF-α) [21]

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10
Q

What is the characteristic Ménétrier disease?

A

The disease is characterized by diffuse hyperplasia of the foveolar epithelium of the body and fundus and hypoproteinemia due to protein-losing enteropathy.

Secondary symptoms, such as weight loss, diarrhea, and peripheral edema, are commonly present.

Symptoms and pathologic features of Ménétrier disease in children are similar to those
in adults, but pediatric disease is usually self-limited and often follows respiratory infection.

Risk of gastric adenocarcinoma is increased in adults with Ménétrier disease.

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11
Q

What is the morphology of Ménétrier disease?

A

is characterized by irregular enlargement of the gastric rugae.

Some areas may appear polypoid.

Enlarged rugae are present in the body and fundus ( Fig. 17-15A ), but the antrum is generally spared.

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12
Q

What is the histological appearance of Ménétrier disease?

A

Histologically, the most
characteristic feature is hyperplasiaof foveolarmucous cells.

The glands are elongated
with a corkscrew-like appearanceandcystic dilation is common ( Fig. 17-15B ).

Inflammation
is usually only modest, although some cases show marked intraepithelial lymphocytosis.
Diffuse or patchy glandular atrophy, evident as hypoplasia of parietal and chief cells, is
typical.

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13
Q
A

FIGURE 17-15 Ménétrier disease.

  • A, Marked enlargement of rugal folds.
  • B, Foveolar hyperplasia with elongated and focally dilated glands
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14
Q

What is the treatment of Ménétrier disease?

A

Treatment of Ménétrier disease is supportive, with intravenous albumin and parenteral nutritional supplementation.

In severe cases gastrectomy may be performed.

More recently, agents that block TGF-α–mediated activation of the epidermal growth factor receptor have
shown promise

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15
Q

What is Zollinger-Ellison syndrome?

A

Zollinger-Ellison syndrome is caused by gastrin secreting tumors, gastrinomas , that are most commonly found in the small intestine or pancreas.

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16
Q

What is the presentation ZOLLINGER-ELLISON SYNDROME?

A

Patients often present with duodenal ulcers
or chronic diarrhea.

17
Q

What is the ZOLLINGER-ELLISON SYNDROME most remarkable feature?

A

Within the stomach, a doubling of oxyntic mucosal thickness due to a fivefold increase in the number of parietal cells .

Gastrin also
induces hyperplasia of mucous neck cells, mucin hyperproduction, and proliferation of endocrine cells within oxyntic mucosa.

In some cases these endocrine cells can form small
dysplastic nodules or, rarely, true carcinoid tumors.

18
Q

What is the treatment for Zollinger-Ellison syndrome?

A

Treatment of individuals with Zollinger-Ellison syndrome includes blockade of acid hypersecretion, which is accomplished in almost all patients with proton pump inhibitors or highdose
H2 histamine receptor antagonists.

Acid suppression allows peptic ulcers to heal and
prevents gastric perforation, allowing treatment to focus on the gastrinoma, which becomes the
main determinant of long-term survival.

19
Q

What are the two
most well-understood examples of Hypertrophic Gastropathies?

A
  • Ménétrier disease and
  • Zollinger-Ellison syndrome
20
Q
A

2H SystemicPatho GIT (34 decks)

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