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. 2022 Dec 16;3(7):62-68.

Early Prophylactic Gastrectomy for the Management of Gastric Adenomatous Proximal Polyposis Syndrome (GAPPS)

A C Salami  1 , J M Stone  2 , R H Greenberg  3 , J C Leighton  2 , R Miick  4 , S R Zavala  5 , K L Zeitzer  6 , C T Bakhos  7   8
Affiliations

Early Prophylactic Gastrectomy for the Management of Gastric Adenomatous Proximal Polyposis Syndrome (GAPPS)

A C Salami et al. ACS Case Rev Surg. .

Abstract

Background: Gastric adenomatous proximal polyposis syndrome (GAPPS) is a recently described, rare, autosomal dominant condition characterized by the extensive involvement of the proximal stomach with hundreds of heterogeneous fundic gland polyps with antral and duodenal sparing. GAPPS is caused by a point mutation of the APC gene promoter 1B and is associated with a risk of malignant transformation, distant metastasis, and death. There are no surveillance, screening, or treatment guidelines for managing GAPPS. The few reported cases have been variably managed with endoscopic surveillance or prophylactic gastrectomy. However, there is no consensus on the optimal management approach.

Summary: In this case series, we review the relevant literature on GAPPS and present two siblings who underwent early prophylactic total gastrectomies with good outcomes.

Conclusion: Due to the poor correlation between the endoscopic findings on sampled polyps and the risk of harboring invasive gastric cancer, patients with GAPPS should be strongly considered for early prophylactic total gastrectomies in the absence of prohibitive comorbidities.

Keywords: GAPPS; gastrectomy; gastric adenocarcinoma; gastric polyposis.

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Conflict of interest statement

DISCLOSURE STATEMENT: The authors have no conflicts of interest to disclose.

Figures

Figure 1.
Figure 1.
Gastric Body—Extensive Polyposis With Heterogenous Polyps. Published with Permission
Figure 2.
Figure 2.
Prepyloric Stomach—Pathognomonic Feature of Antral Sparing. Published with Permission
Figure 3.
Figure 3.
Gastric Fundic Mucosa With Numerous Fundic Gland Polyps With Hyperplastic Aberrant Pits at the Luminal Surfaces (H&E, 20x). Published with Permission
Figure 4.
Figure 4.
Hyperplastic Aberrant Pits (Proliferation of the Foveolar Pits, Resulting in Foveolar Pit Crowding) and Surface Epithelium With High-Grade Dysplasia, Demonstrating Full-Thickness Nuclear Stratification With Loss of Nuclear Polarity (H&E, 600x). Published with Permission
Figure 5.
Figure 5.
Pedigree Chart
See this image and copyright information in PMC

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References

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