INTRODUCTION
Pyloric gland adenoma (PGA) is a rare precancerous neoplasm of the gastric mucosa with a reported transformation rate to adenocarcinoma of 12%–47%.1–4 It typically presents as an isolated polypoid lesion in the stomach, but has also been observed in the duodenum, gallbladder, pancreas, rectum, and esophagus.5–8 There is currently no established guideline on the management of PGA, but resection is recommended because of its malignant potential.4 Endoscopic submucosal dissection (ESD) has been reported as a viable strategy for the treatment of a single lesion in the stomach or esophagus.8,9 However, this has not been described when the adenoma presents in a diffuse, circumferential pattern. We present a case of multifocal, circumferential PGA within long-segment Barrett's esophagus treated successfully with ESD.10
CASE REPORT
A 49-year-old White, overweight woman with active tobacco use presented complaining of pyrosis and regurgitation. She reported a history of gastroesophageal reflux disease and was adherent to twice-daily esomeprazole, but continued to have breakthrough symptoms.
Esophagogastroduodenoscopy (EGD) demonstrated endoscopic evidence of Barrett's esophagus, C10M14, with extensive carpeted nodularity throughout the salmon-colored mucosa within the tubular esophagus with circumferential involvement at the gastroesophageal junction with extension to the cardia. Examination under high-definition white light and narrow-band imaging showed areas of a tortuous, dilated pit pattern without ulceration or evidence of malignancy (Figures 1 and 2).
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Figure 1.: Mid-esophagus on high-definition white light showing carpeted nodularity in the background of Barrett's esophagus.
Figure 2.: Mid-esophagus on narrow-band imaging with nodules demonstrating a dilated, tortuous pit pattern.
Given concern for dysplasia and the extensive involvement of the disease, targeted biopsies were obtained throughout the esophagus to delineate the extent of mucosal abnormality and to evaluate for dysplasia. Pathology showed columnar mucosa with intestinal metaplasia and multifocal PGA without dysplasia extending circumferentially and throughout the length of Barrett's esophagus. She was deemed a poor surgical candidate for esophagectomy and referred for endoscopic submucosal resection.
Equipment used include an Olympus 190 gastroscope, CO2 insufflation, 500 mL hetastarch mixed with 10 mL of 1% methylene blue, Orise, 1.5 mL DualKnife J, ClutchCutter, a ERBE VIO 200D electrosurgical unit, 11 mL standard through-the-scope clips, and suture material. The technique of ESD included marking the oral and anal sides, injection and circumferential incision within the cardia of the stomach with the 1.5 mm DualKnife J, creation of 3 submucosal tunnels with an interconnecting septum, followed by dissection of the septum with the ClutchCutter. Three 11 mm clip-with-line method was used to apply traction to the 3 tunnels. The patient underwent successful circumferential ESD with prophylactic steroid injection to prevent stricture formation (Figure 3).
Figure 3.: Mid-esophagus status post successful endoscopic submucosal dissection with hemostasis.
Pathologic examination confirmed a diffuse and multifocal PGA in a background of extensive intestinal metaplasia without evidence of dysplasia and negative margins. The patient reported mild dysphagia, with follow-up endoscopy demonstrating mild luminal stenosis treated with serial dilations with improvement of the patient's symptoms. Surveillance biopsies were negative for recurrence.
PGA is an uncommon gastrointestinal lesion with a significant malignant potential. It was first described by Kurt Elster in 1976 and was included in the World Health Organization classification of gastric tumor in 1990. In the largest case series published to date, PGAs are found to predominantly affect female patients in a 3:1 ratio with a mean age of diagnosis of 73 years.2 Most PGAs are found in the stomach, with predilection for corpus mucosa, particularly in those with autoimmune gastritis.2
Encounter of PGA in extragastric sites including the esophagus, duodenum, gallbladder, and pancreas have been reported, mostly related to pyloric glandular metaplasia.5–8 In the esophagus, PGA may arise in either Barrett's mucosa or normal epithelium.6 Endoscopically, under white light, most PGAs appear as polypoid or mass-like lesions, although presentation as flat, irregular mucosa; ulceration; or subepithelial lesion has been reported. PGAs present with large lesions at the time of diagnosis, measuring 1.6–2.3 cm on average, and may demonstrate dimpling on the surface of the protuberant lesion, which should be distinguished from ectopic pancreas or neuroendocrine tumors.9,12
Histologically, PGA consists of tightly packed pyloric glands lined by cuboidal or low columnar epithelium with ground-glass eosinophilic cytoplasm, round basally located nuclei with inconspicuous nucleoli, and absent apical mucin (Figure 4).2,5,11,13 Although immunohistochemistry is not required to diagnose PGAs, it may be useful in cases when there is diagnostic uncertainty. PGAs can be confirmed immunohistochemically by staining positively for gastric mucin apoprotein 6 (MUC6) and MUC5AC. MUC6 expression is diffusely and strongly positive throughout the lesion and its surface, with high sensitivity and specificity, whereas MUC5AC has low or lost expression in the surface epithelium (Figure 5).2,9,13
Figure 4.: Tightly packed pyloric glands lined by cuboidal or low columnar epithelium with ground-glass eosinophilic cytoplasm, round basally located nuclei with inconspicuous nucleoli, and absent apical mucin. The arrow points to a representative pyloric gland (10× magnification).
Figure 5.: Gross specimen of circumferential endoscopic submucosal dissection of the esophagus measuring 13.9 cm in length affixed to a plastic tube.
While there is no guideline on the management of PGA, complete excision is recommended because of its high risk of malignant transformation into adenocarcinoma, from 12% to 47%.1–4 In a recent multicenter clinicopathologic case series, PGAs with high-grade dysplasia or adenocarcinoma were found to have larger mean size (3.5 vs 1.5 cm), tubulovillous architecture, or coexpression of both MUC6 and MUC5AC in deeper glands.2 After endoscopic or surgical resection, PGAs have a low local recurrence rate, less than 10%.2 Of note, there is no current guidance on surveillance EGDs for PGAs after endoscopic resection. Because the local recurrence rate of PGAs is comparable with the recurrence rate of dysplasia after endoscopic eradication therapy is performed for Barrett's esophagus-related neoplasia (0%–15%), we elected to perform surveillance EGDs every 3 months for the first year, every 6 months for year 2, and annually thereafter, with biopsy of visible mucosal abnormalities.14,15
This case poses a unique, challenging clinical conundrum owing to the extensive involvement of the lesion. To our knowledge, this is the first reported case of a circumferential, multifocal PGA in the esophagus. We demonstrated that ESD is a feasible and safe management option for select patients in whom interval repeat surveillance EGDs can be performed with minimal postprocedural complications, which can be managed with endoscopy. PGAs are clinically significant with a high malignant potential, and with improved understanding in the molecular mechanisms involved in the neoplastic transformation of these lesions, gastroenterologists and pathologists can become more familiar with this entity and improve its management.
DISCLOSURES
Author contributions: HD Lee conceptualized and drafted the work, is accountable for all aspects of the work, and is the article guarantor. JP Magulick and JG Quiles conceptualized and critically revised the work and provided final approval of the version.
Financial disclosure: None to report.
Previous presentation: Poster presentation: ACG 2022 Annual Scientific Meeting & Postgraduate Course; October 25, 2022; Charlotte, North Carolina.
Informed consent was obtained for this case report.
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