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Diffuse (poorly cohesive) type adenocarcinoma

Reviewers: Elliot Weisenberg, M.D. (see Reviewers page)
Revised: 29 May 2013, last major update August 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.


● Infiltrative growth of poorly differentiated discohesive malignant cells
● Also called linitis plastica or signet ring adenocarcinoma (defined as predominantly or exclusively signet ring cells)
● Mean age 48 years, M=F
● More common in young; incidence unchanged over past 50 years (in contrast to reduction in intestinal type)
● In US in 2012, similar incidence as intestinal type
● Appears to arise from different pathway than intestinal type (Am J Surg Pathol 1996;20(S1):S8)
● No known risk factors except rarely hereditary diffuse gastric cancer (rare, autosomal dominant, related to mutations in E-cadherin gene (CDH1); may have early "in situ" component

Case reports

● 35 year old woman exposed to Chernobyl explosion with upper abdominal pain (Case of the Week #275)

Gross description

● Linitis plastica: broad region of gastric wall or entire stomach is extensively infiltrated by malignancy, creating a thickened, rigid, leather bottle-like stomach; may cause pyloric obstruction

Gross images

Various images

Micro description

● Gastric-type mucus cells, usually do NOT form glands, infiltrate as individual cells or small clusters, may be transmural
● Appear to arise from middle layer of mucosa
● Intestinal metaplasia usually not present
● Numerous signet ring cells seen (mucin pushes nucleus to periphery)
● Submucosal fibrosis present, variable mucosal ulceration
● Hypertrophic muscularis propria
● May have marked desmoplastic and inflammatory reaction

Micro images

Various images

Case of the Week #275:
Left to right: H&E, CAM 5.2, CEA

Positive stains

● Mucicarmine, Alcian blue-PAS, CEA, EMA, keratin, villin

Negative stains

● TTF1, p53 (usually)

Differential diagnosis

● Lymphoma with artifactual signet ring cells due to cytoplasmic shrinkage (Arch Pathol Lab Med 1997;121:623)
● Metastases from breast (Arch Pathol Lab Med 2001;125:567), lung
● Reactive epithelial atypia associated with chemotherapy or otherwise
● Xanthoma

End of Stomach > Carcinoma > Diffuse (poorly cohesive) type adenocarcinoma

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