Hereditary Diffuse Gastric Cancer
Hereditary diffuse gastric cancer (HDGC) is caused by germline CDH1 (~25%) or CTNNA1 (~2-5%) mutations; diffuse/signet ring histology; lifetime GC risk ~83% (CDH1 male); prophylactic gastrectomy is recommended; lobular breast cancer risk is elevated in CDH1/CTNNA1 carriers.
Entry Metadata
| Field | Value |
|---|---|
| ID | hereditary-diffuse-gastric-cancer |
| Name | Hereditary Diffuse Gastric Cancer |
| Status | draft |
| Last reviewed | 2026-06-07 |
| Atlas | 01-human |
| Scale | 07-system |
Cross-Atlas Connections
Sources
- van der Post RS, Vogelaar IP, Carneiro F, et al. Hereditary diffuse gastric cancer: updated clinical guidelines with an emphasis on germline CDH1 mutation carriers. J Med Genet. 2015;52(6):361-374. · PubMed 25979631
- Hansford S, Kaurah P, Li-Chang H, et al. Hereditary Diffuse Gastric Cancer Syndrome: CDH1 Mutations and Beyond. JAMA Oncol. 2015;1(1):23-32. · PubMed 26182300