Hereditary diffuse gastric adenocarcinoma
- Synonyms
- Hereditary diffuse gastric cancer
- Modes of inheritance
- Autosomal dominant inheritance (Orphanet)
Summary
Excerpted from the GeneReview:Diffuse gastric and lobular breast cancer syndrome (DGLBCS) is associated with an increased risk, in males and females, of diffuse gastric cancer (DGC), a poorly differentiated adenocarcinoma (also referred to as signet ring cell carcinoma or isolated cell-type carcinoma) that infiltrates into the stomach wall, causing thickening of the wall (linitis plastica) without forming a distinct mass. In females with CDH1-related DGLBCS, but not in males, there is also an increased risk for lobular breast cancer (LBC), characterized by small, non-cohesive cells dispersed in the stroma or arranged in single-file infiltrating patterns. Cleft lip with or without cleft palate has also been reported in some individuals with CDH1-related DGLBCS.
- Full text of GeneReview (by section):
- Summary
- Diagnosis
- Clinical Characteristics
- Genetically Related (Allelic) Disorders
- Differential Diagnosis
- Management
- Genetic Counseling
- Resources
- Molecular Genetics
- Chapter Notes
- References
- Authors:
- Rita Barbosa-Matos
- Lilian Córdova
- Kasmintan Schrader
- view full author information
Available tests
171 tests are in the database for this condition.
Check Related conditions for additional relevant tests.
Clinical features
Help- Abnormality of head or neck
- Cleft palate
Cleft palate
- MedGen UID: 756015
- Concept ID: C2981150
- Finding: Congenital Abnormality
Abnormality of head or neck
- Cleft upper lip
Cleft upper lip
- MedGen UID: 40327
- Concept ID: C0008924
- Finding: Congenital Abnormality
Abnormality of head or neck
- Cleft palate
- Abnormality of the digestive system
- Atrophic gastritis
Atrophic gastritis
- MedGen UID: 42157
- Concept ID: C0017154
- Finding: Disease or Syndrome
Abnormality of the digestive system
- Gastric cancer
Gastric cancer
- MedGen UID: 44264
- Concept ID: C0024623
- Finding: Neoplastic Process
Abnormality of the digestive system
- Atrophic gastritis
- NCI PDQ, Breast and Gynecologic CancersGenetics of Breast and Gynecologic Cancers (PDQ®): Health Professional Version
- NCI PDQ, Cancer Genetics CounselingCancer Genetics Risk Assessment and Counseling (PDQ®): Health Professional Version
- NCCN, 2023NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) Gastric Cancer, 2023
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