Journal: Annals of surgical oncology
This study retrospectively analyzed 21 patients with gastric adenocarcinoma and proximal polyposis of the stomach (GAPPS), a rare hereditary gastric cancer syndrome linked to APC promoter IB variants.
The median age at genetic testing was 44 years, with most patients undergoing esophagogastroduodenoscopy (EGD) around age 37.
All patients showed gastric polyposis primarily in the fundus and body, sparing the antrum.
Endoscopic biopsies revealed:
- Gastric adenocarcinoma
- High- and low-grade dysplasia
- Benign fundic gland polyposis
Nearly half of the patients (43%) underwent total gastrectomy, which often revealed more advanced disease than biopsy samples suggested.
Due to the challenges of surveillance and the risk of cancer, prophylactic total gastrectomy is recommended.
However, further longitudinal studies are needed to refine management guidelines.