Gastric adenocarcinoma and proximal polyposis of the stomach (GAPPS) is an autosomal-dominant
syndrome developing gastric carcinoma with a background of fundic gland polyposis
[1]. Although prophylactic total gastrectomy is considered given the high incidence
of gastric cancer [2]
[3], there is no consensus/guideline. We report a case of treating a pyloric gland adenoma
(PGA) in GAPPS with endoscopic submucosal dissection (ESD).
A 54-year-old woman was referred to our hospital with a diagnosis of fundic gland
polyps (FGPs). FGPs were localized in the gastric body and fundus ([Fig. 1]), but there were no polyps in the antrum and duodenum. A 20-mm white elevated lesion
was observed in the greater curvature of the upper body. Under narrow-band-imaging
magnifying endoscopy, arcuate glandular duct structures were observed, and the demarcation
line could be identified ([Fig. 2]). She had several relatives with gastric cancer; particularly her brother was diagnosed
with fundic gland polyposis. Hence, we suspected a gastric-type tumor associated with
GAPPS.
Fig. 1 Endoscopic findings of the stomach (distant view). Fundic gland polyps were observed
in the gastric body and fundus, except for the lesser curvature.
Fig. 2 Endoscopic findings of the tumor. A 20-mm white elevated lesion was observed in the
greater curvature of the upper body.
ESD was performed because the patient refused the surgery ([Video 1]). The lesion was resected en bloc using the clip-and-thread traction method ([Fig. 3]). The lesion consisted of closely packed neoplastic glands resembling pyloric glands.
The tumor exhibited pronounced cytological and architectural atypia in some areas
([Fig. 4]). Immunohistochemistry revealed diffuse reactivity to MUC6 and focal reactivity
to MUC5AC. The histological diagnosis was PGA with high grade dysplasia.
Video 1 A case of treating a pyloric gland adenoma in gastric adenocarcinoma and proximal
polyposis of the stomach with endoscopic submucosal dissection.
Fig. 3 Procedure of endoscopic submucosal dissection. The lesion was resected en bloc by
the clip-and- thread traction method.
Fig. 4 Histopathological findings (hematoxylin and eosin staining). a The main lesion was a pyloric gland adenoma (PGA) showing a flat elevation. Two fundic
gland polyps were adjacent to PGA. b The PGA was composed of pyloric-type glands. c High grade atypia was noted.
Genome analysis of the APC gene using peripheral blood demonstrated a point mutation c.-191T > C in exon 1B,
a characteristic mutation of GAPPS [4]. The final diagnosis was pyloric gland adenoma associated with gastric adenocarcinoma
and proximal polyposis of the stomach.
Although the effectiveness of endoscopic surveillance is unestablished [2], considering the high risk of carcinogenesis in the residual stomach, close endoscopic
follow-up is planned. It is interesting that a white patch in fundic gland polyps
as observed in this case is associated with a high rate of proximal gastric cancer
in familial polyposis [5].
Endoscopy_UCTN_Code_CCL_1AB_2AD_3AB
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