GPR: 2021 - Week 02

From MGH Learn Pathology

Clinical history

42-year-old male with multiple episodes of upper gastrointestinal bleeding.


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Ménétrier disease
  • 5th-6th decades; M > F
  • May present with upper GI bleeding
  • Increased gastric mucous secretions on endoscopy
  • Hyperplastic gastric folds up to several cm in thickness
  • Foveolar hyperplasia with fluffy appearance
  • Predominantly involves fundus
  • Ulcerations may be present
  • Driven by overexpression of TGF-α
  • Association with CMV (pediatric cases), H. pylori
 

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Juvenile polyposis syndrome

  • Presents with GI bleeding
  • Numerous juvenile polyps (>5 per diagnostic criteria)
  • Polyps throughout GI tract (rarely, may be confined to stomach)
  • Variable size; most polyps ~ 1.0 cm
  • Polyps are predominantly pedunculated
  • Gelatinous, mucin-filled cysts may be present
  • 40% driven by autosomal dominant mutation in SMAD4 (TGF-β signaling)
  • Associated with AVMs and HHT
  • Increased gastric, duodenal, colorectal cancer risk
 

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Hereditary diffuse gastric cancer

  • Rare (~1-3% of gastric cancers)
  • ~4th - 5th decade
  • Lesions not grossly appreciated in early disease
  • Linitis plastica in advanced disease
  • Presents with pain, obstructive symptoms
  • Upper GI bleeding uncommon
  • Autosomal dominant mutation in CDH1
  • ~40-83% lifetime risk of diffuse gastric cancer
 

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Gastrointestinal stromal tumor

  • > 6th decade
  • Commonly presents with GI bleeding
  • Ulceration may be present
  • Well-circumscribed
  • Originates in the muscularis propria
  • Fleshy cut surface
  • May be cystic, focally hemorrhagic, focally necrotic
  • Stomach is most common site
  • Up to 35% of general population have <1 cm clinically silent “micro-GIST”
  • Driven by ligand-independent KIT activation
 
Discussion:
  • Dr. Lawrence Zukerberg

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Gross photographs:

  • Kara Tassinari
  • Samuel Benson
  • Stefanie Flores

This week's Gross Pathology Roundup was presented by Soma Jobbagy, MD, PhD on Mon, Jan 11, 2021.