Filling in the “GAPPS”: an unusual presentation of a child with gastric adenocarcinoma and proximal polyposis of the stomach

Grossman, Arielle; Colavito, John; Levine, Jeremiah; Thomas, Kristen; Greifer, Melanie

doi:10.1007/s10120-021-01249-7

Cited by 8 publications

(14 citation statements)

References 10 publications

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“…GAPPS was first reported by Worthly et al in 2012 [ 1 ]. Since then, there have been an increasing number of reports on GAPPS [ 5 – 9 ]; however, the clinical course, malignant transformation, and prognosis of GAPPS remain unclear. The prognosis of GAPPS depends on the malignancy of the FGPs.…”

Section: Discussionmentioning

confidence: 99%

Four cases of gastric adenocarcinoma and proximal polyposis of the stomach treated by robotic total gastrectomy

et al. 2022

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Background Gastric adenocarcinoma and proximal polyposis of the stomach (GAPPS) is a rare disease and characterized by a unique point mutation in the promoter 1B region of the adenomatous polyposis coli (APC) gene. There are two aims in surgery for GAPPS; the first is prophylactic gastrectomy, and the second is excising concurrent cancer. We performed robotic total gastrectomy (RTG) for four cases of GAPPS. Case presentation Case 1 was a woman in her 40 s whose sister had died from gastric cancer. Mutational analysis revealed mutation of APC exon 1B. We performed prophylactic gastrectomy. Case 2 was a woman in her 30 s who had a mutation of APC exon 1B, and preoperative biopsy revealed suspected adenocarcinoma. Case 3 was a woman in her 40 s who was diagnosed with gastric cancer with multiple polyps in the stomach and a mutation of APC exon 1B. Case 4 was a woman in her 20 s in whom biopsy revealed low-grade dysplasia of a raised lesion. She had a mutation in APC exon 1B. We performed RTG with D1 + lymphadenectomy in all patients, and there were no intraoperative complications. Conclusions Patients with GAPPS are mainly followed regularly with repeat biopsy, and tumors are detected in an early stage. As the safety of robotic surgery for the early gastric cancer is reported, RTG is an option for these patients. This is the first report of RTG for GAPPS patients.

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Section: Discussionmentioning

confidence: 99%

Four cases of gastric adenocarcinoma and proximal polyposis of the stomach treated by robotic total gastrectomy

et al. 2022

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“…15 In early reports, the death of a family member was the main reason for surgery without a genetic test, but in recent reports, preoperative genetic tests are performed to diagnose GAPPS, and prophylactic total gastrectomy has been performed. 14,16,[19][20][21][22] With the spread of minimally invasive surgery, laparoscopic or robotic-assisted total gastrectomy has become popular. 14,16,21 Iwaoka et al 16 reported from their experience with four GAPPS cases treated by robotic total gastrectomy that there were a large number of polyps, but there was no problem in grasping the stomach wall with forceps.…”

Section: Prophylactic Gastrectomymentioning

confidence: 99%

“…Total gastrectomy with lymph node dissection is required if GAPPS has already developed GC without distant metastasis. Table 1 summarizes the cases of therapeutic and prophylactic gastrectomy for GAPPS 5,8,10,14,16–22 . On the other hand, if not already developed with GC, prophylactic total gastrectomy for GAPPS must be considered very carefully, with caution, and with a fully individualized approach.…”

Section: Treatmentmentioning

confidence: 99%

“…Furthermore, Foretova et al 19 recommended prophylactic total gastrectomy for cases with progressive massive stomach polyposis even without dysplasia. Table 1 summarized the cases who underwent prophylactic total gastrectomy for GAPPS 8,14,16,19–22 . Based on these previous reports, the algorithm for GAPPS family members with or without mutation of the 1B promoter of the APC gene is proposed 15 .…”

Section: Treatmentmentioning

confidence: 99%

“…Based on these previous reports, the algorithm for GAPPS family members with or without mutation of the 1B promoter of the APC gene is proposed 15 . In early reports, the death of a family member was the main reason for surgery without a genetic test, but in recent reports, preoperative genetic tests are performed to diagnose GAPPS, and prophylactic total gastrectomy has been performed 14,16,19–22 . With the spread of minimally invasive surgery, laparoscopic or robotic‐assisted total gastrectomy has become popular 14,16,21 .…”

Section: Treatmentmentioning

confidence: 99%

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The comprehensive review of gastric adenocarcinoma and proximal polyposis of the stomach (GAPPS) from diagnosis and treatment

Iwatsuki

Matsumoto

Mimori

et al. 2023

Annals of Gastroent Surgery

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Gastric adenocarcinoma and proximal polyposis of the stomach (GAPPS) was first proposed by Wothley et al. in 2012 as a rare familial gastric cancer syndrome associated with an autosomal dominant form of inheritance. GAPPS is characterized by gastric basal gland polyposis from the hilum to the body of the stomach. Li et al. in 2016 showed that the cause of the disease is a point mutation in the promotor 1B region of the APC gene, and genetic testing was used to confirm the diagnosis. If the patient has already developed gastric cancer, treatment should be based on the usual treatment for gastric cancer. If no distant metastases exist, a good prognosis can be expected by performing a total gastrectomy. On the other hand, patients with distant metastasis have a poor prognosis. In the case of dysplasia, prophylactic total gastrectomy is recommended, but because it is highly invasive and postoperative postgastrectomy syndrome must be considered, the decision should be made with careful consideration of the patient's background. Therefore, there are no guidelines for screening for GAPPS, the timing of prophylactic total gastrectomy, or methods of endoscopic surveillance. Because GAPPS is a rare disease, its natural history is still unclear. Further case series are needed to elucidate the molecular biology and clinicopathological features of GAPPS and to establish clinical management, including diagnosis, treatment, and surveillance. In this review, we provide an overview of GAPPS, its clinical management, and its problems, which will be useful for the treatment of GAPPS.

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Epithelial tumours of the stomach

Kővári,

Carneiro,

Lauwers

2024

Morson and Dawson's Gastrointestinal Pathology

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Filling in the “GAPPS”: an unusual presentation of a child with gastric adenocarcinoma and proximal polyposis of the stomach

Cited by 8 publications

References 10 publications

Four cases of gastric adenocarcinoma and proximal polyposis of the stomach treated by robotic total gastrectomy

Four cases of gastric adenocarcinoma and proximal polyposis of the stomach treated by robotic total gastrectomy

The comprehensive review of gastric adenocarcinoma and proximal polyposis of the stomach (GAPPS) from diagnosis and treatment

Epithelial tumours of the stomach

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