Stomach disorders Flashcards
Risk factors for congenital hypertrophic pyloric stenosis
Down and Turner syndromes
Trisomy 18
Erythromycin or azithromycin (PO or in breastmilk)
Concentric hyperplasia and hypertrophy caused by fibrosis and elastosis of pyloric muscularis that is sometimes associated with mucosal erosions and inflammation
Congenital hypertrophic pyloric stenosis
4 wk old infant is brought in within new-onset regurgitation, projectile non-bilious vomiting after feeding, and frequent demands for refeeding. A firm, ovoid, 1-2 cm abdominal mass is palpated on exam.
Congenital hypertrophic pyloric stenosis
Curative treatment for hypertrophic pyloric stenosis
Myotomy –> surgical splitting of muscularis
Causes of acquired hypertrophic pyloric stenosis
Antral gastritis or peptic ulcer close to pylorus
Carcinomas of distal stomach and pancreas
Fibrosis or malignant infiltration
Finding to differentiate acute gastritis from gastropathy
Presence of neutrophils
Inflammation of the gastric mucosa with associated intestinal metaplasia and atrophy. Lymphocytes and plasma cells are present
Chronic gastritis
Signs and symptoms of more severe gastritis with mucosal erosion/ulceration
Hemorrhage
Hematemesis
Melena
Massive blood loss (rare)
Gross appearance of erosive gastritis
Mucosal denudation
Gross appearance of hemorrhagic gastritis
Dark puncta within hyperemic mucosa
Gastric biopsy shows intact surface epithelium with foveolar cell hyperplasia and corkscrew profiles of epithelial proliferation. Neutrophils are present among the epithelial cells and within mucosal gland lumina. Moderate edema and slight vascular congestion of the lamina propria is also present.
Reactive gastropathy/gastritis
Abrupt onset of abdominal pain and bleeding with multiples erosions in the gastric mucosa associated with trauma.
Stress-related mucosal disease
Features of curling ulcers in stress-related mucosal disease
Proximal duodenal ulcers
Associated with severe burns
Features of Cushing ulcers in stress-related mucosal disease
Gastric, duodenal, and esophageal ulcers with an increased risk of perforation.
Associated with intracranial disease
Multiple, small, rounded gastric ulcers with a brown to black base due to acid digestion of extravasation of blood. Microscopy is absent for scarring and blood vessel thickening.
Stress-related mucosal disease
Characterized by the presence of chronic mucosal inflammatory changes that may lead to mucosal atrophy and intestinal metaplasia. Associated with increased risk of carcinoma.
Chronic gastritis
Most common cause of chronic gastritis
H pylori infection
Systemic diseases associated with chronic gastritis
Crohn’s disease
Amyloidosis
GVHD
Predominantly causes antral gastritis. Associated with gastric and/or duodenal peptic ulcer disease. Can lead to atrophic gastritis.
H pylori infection
Complications of H pylori chronic gastritis
Gastric adenocarcinoma –> atrophy
Duodenal ulcers
B cell lymphoma –> lymphoid aggregates
Chronic gastritis type that spares the antrum and is associated with hypergastrinemia
Autoimmune
CD4+ T cells against parietal cell components leading to loss of parietal cells and decreased or absent secretion of gastric acid and intrinsic factor
Autoimmune gastritis
Gastric biopsy shows diffuse mucosal damage of the oxyntic mucosa within the body and fundus. Mucosa is thinned and there is loss of rugae.
Autoimmune gastritis
Distinctive endoscopic appearance of the stomach characterized by thickened folds covered by small nodules with central aphthous ulceration
Varioliform gastritis, associated with lymphocytic gastric
Gastric mucosal atrophy is generally protective against what?
Antral and duodenal ulcers
Ulcers on greater curvature of the stomach are associated with what etiology?
NSAID use
Biopsy of gastric ulcer shows superficial zone of fibrinopurulent exudate, necrotic tissue, granulation tissue, and fibrotic tissue with chronic inflammation in base with thrombosed vessel.
Chronic peptic ulcer
Complications of PUD
Obstruction
Bleeding
Perforation
Uncommon diseases of giant cerebriform enlargement of the rugal folds due to epithelial hyperplasia without inflammation. Linked to excessive growth factor.
Hypertrophic gastropathies
Types of hypertrophic gastropathies
Menetrier disease
Zollinger-Ellison syndrome
Rare acquired disorder involving the gastric body that is associated with gastric rugal hypertrophy, protein-losing gastropathy, and hypochlorhydria. Associated with CMV or H pylori infection.
Menetrier disease
Causes of Menetrier disease
Increased TGF-alpha
Hyperactivation of EGFR on gastric epithelial cells
Endoscopy shows irregular enlargement of the gastric rugae in body and fundus with sparing of antrum. Biopsy shows hyperplasia of foveolar mucous cells, elongated corkscrew-like glands, and cystic dilation with glandular atrophy and hypoplasia of the parietal and chief cells.
Menetrier disease
Manifestation of hypoproteinemia in Menetrier disease
Peripheral edema
Menetrier disease in children
Self-limited
Associated with CMV infection
Triad of Zollinger-Ellison syndrome
Hypergastrinemia
Increased acid production
PUD
Cause of hypergastrinemia in Zollinger-Ellison syndrome
Gastrin-secreting neuroendocrine tumors
Condition associated with formation of gastrinomas leading to Zollinger-Ellinger syndrome at a younger age
Multiple endocrine neoplasia type 1 (MEN1)
Endoscopy shows massive hypertrophy of the gastric rugae in the body and fundus with reduction of the antral mucosa. Biopsy shows hypertrophy and hyperplasia of Parietal cells, which extend up to the foveolar neck region and to the gastric antrum.
Zollinger-Ellison syndrome
Main determinant of long term survival in Zollinger-Ellison syndrome
Gastrinoma
Adenocarcinoma type in stomach that forms bulky masses
Intestinal type
Adenocarcinoma type in stomach that infiltrates and thickens the gastric wall
Diffuse type
Precursor lesions of intestinal type gastric adenocarcinoma
Flat dysplasia
Adenomas
Mutation in familial gastric cancer
Loss of function in CDH1, a tumor suppressor that codes E-cadherin
Mutations associated with intestinal type gastric adenocarcinoma
Increased signaling in Wnt pathway
APC
Beta-catenin
TGF-beta signaling
BAX
CDKN2A
Key step in the development of diffuse type gastric adenocarcinoma
E-cadherin loss –> loss of function of CDH1 in 50%
Endoscopy reveals ulcer with heaped up margin and loss of spoke wheel pattern
Malignancy –> gastric adenocarcionma
Features of linitis plastica
Diffuse rugal flattening and rigid, thickened wall
Associated with diffuse type of gastric adenocarcinoma
Malignant gastric ulcer present on endoscopy. Gastric biopsy shows atypical glandular structures with apical mucin vacuoles, abundant mucin in glandular lumina, and infiltrating irregular glands of columnar cells with high N:C ratio and irregular nuclear membrane.
Intestinal type gastric adenocarcinoma
Endoscopy shows malignant gastric ulcer. Gastric biopsy shows signet ring cells with large mucin vacuole.
Diffuse type gastric adenocarcinoma
Paraneoplastic syndromes associated with gastric adenocarcinoma
Acanthosis nigricans
Leser-Trelat sign
Metastasis sites of gastric adenocarcinoma
Supraclavicular and periumbilical lymph nodes
Ovaries
L axillary lymph nodes
Pouch of Douglas
5 yr survival rate of gastric adenocarcinoma in US
<30% due to advanced stage at diagnosis
Most common type of gastric lymphoma/MALToma
Extra-nodal marginal zone B cell lymphoma
Translocations associated with gastric lymphoma/MALToma
t(11;18)(q21;q21) –> API2-MALT1 fusion protein (common)
t(1;14)(p22;q32) –> BCL-10
t(14;18)(q32;21) –> MALT1
Endoscopy shows ulcerated, polypoid, granulonodular, erythematous, edematous gastric folds. Biopsy shows monotonous infiltrate of monocytoid cells mixed with immunoblasts and plasmacytoid cells. Infiltrate expands the lamina propria and reactive follicles are often present
Gastric MALToma
5 year survival rate of gastric MALToma
50-90%
Most common mesenchymal tumor of the abdomen
GIST
Syndrome with increased risk of developing GIST
NF1
Nonhereditary Carney triad
GIST
Paraganglioma
Pulmonary chondroma
Mutations associated with GIST
Gain of function in cKIT
PDGFR-alpha
Metastasis of GIST
Multiple serosal nodules throughout peritoneal cavity and/or within liver
Spread outside abdomen is uncommon
Excised solitary, well-circumscribe, fleshy mass from stomach. Cut surface has a whorled appearance. Microscopy shows thin, elongated cells that are cKIT positive.
GIST
Classifications of GIST
Spindle cell type
Epitheloid cell type
Mixed
Targeted therapy for tumors with mutations in KIT or PDGFR-alpha that can be used in unresectable GIST
Imatinib
Gastric neuroendocrine neoplasm with endocrine cell hyperplasia is associated with what?
Autoimmune chronic atrophic gastritis
Gastric neuroendocrine neoplasm of MEN1 is associated with what?
Zollinger-Ellison syndrome
Yellow or tan intramural or submucosal gastric masses that create polypoid lesion and typically arise within the oxyntic mucosa. Microscopy shows round to oval nuclei with salt and pepper chromatin.
Gastric neuroendocrine tumor/gastric carcinoid
Symptoms of carcinoid syndrome
Cutaneous flushing
Sweating
Bronchospasm
Colicky abdominal pain
Diarrhea
Cause of carcinoid syndrome
Vasoactive substances released by neuroendocrine tumors
Complication of carcinoid syndrome
R-sided cardiac valvular fibrosis