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

Abstract

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.

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

“…Table 1 summarizes the cases of therapeutic and prophylactic gastrectomy for GAPPS. 5,8,10,14,[16][17][18][19][20][21][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. Repak et al 10 stated that patients who fulfill the original GAPPS criteria and those with FGP progression to dysplasia should be tested for genetic mutations so that prophylactic gastrectomy can be performed appropriately.…”

“…Table 1 summarized the cases who underwent prophylactic total gastrectomy for GAPPS. 8,14,16,[19][20][21][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 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.…”

“…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.…”

“…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.…”

“…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 .…”