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Esophageal Adenocarcinoma with Enteroblastic Differentiation arising in Ectopic Gastric Mucosa in the Cervical Esophagus: a Case Report and Literature Review

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Ryosuke Gushima1, Rei Narita1, Takashi Shono1, Hideaki Naoe1, Takashi Yao2, Yutaka Sasaki1
1) Department of Gastroenterology and Hepatology, Graduate School of Medical Sciences, Kumamoto University, 1-1-1, Honjo, Chuo-ku, Kumamoto 860-8556;
2) Department of Human Pathology, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo 113-8421, Japan

DOI: http://dx.doi.org/10.15403/jgld.2014.1121.262.gus

Background: Adenocarcinoma with enteroblastic differentiation is a subtype of alpha-fetoprotein (AFP) producing adenocarcinoma. This type of tumor is associated with a poor prognosis and is prone to metastasize. Esophageal adenocarcinoma with enteroblastic differentiation is extremely rare.
Case presentation: The patient was a 65-year-old woman who was referred to our hospital with dysphagia. Endoscopic examination revealed an elevated lesion 20mm in diameter at 17cm from the upper incisors. Endoscopic submucosa dissection (ESD) was performed and histopathological examination revealed tubular adenocarcinoma composed of cuboidal cells with clear cell cytoplasm. Immunohistochemical stain was diffusely positive for Sall-like protein 4 (SALL4) and weakly positive for AFP and glypican 3. From this result, we diagnosed esophageal adenocarcinoma with enteroblastic differentiation. The patient is still alive without recurrence of cancer 40 months after ESD.
Conclusion: To our knowledge, this is the first report to undergo ESD for esophageal adenocarcinoma with enteroblastic differentiation arising from ectopic gastric mucosa in the esophagus.
Key words: Esophageal adenocarcinoma – enteroblastic type –AFP – SALL4 – glypican 3.
Abbreviations: AFP: alfa-fetoprotein; CA19-9: carbohydrate antigen 19-9; CEA: carcinoembryonic antigen; ESD: endoscopic submucosal dissection; EUS: endoscopic ultrasound; FDG-PET: [18F] fluorodeoxyglucose positron emission tomography; ME: magnifying endoscopy; NBI: narrow band imaging; SALL 4: Sall-like protein 4; SCC: squamous cell carcinoma antigen.