Glandular heterotopia of the upper esophagus is a congenital abnormality that is frequently discovered during upper digestive tract endoscopy (in 0.26-4.9% of cases), but usually with no malignant potential. Indeed, adenocarcinoma of the upper esophagus related to such lesions is a rare entity. We report here the 27th observation of this rare type of tumor in an 87-year-old man complaining of cervical dysphagia. This adenocarcinoma had developed from an area of gastric heterotopia 3 cm below the upper sphincter of the esophagus. The treatment regimen was based on exclusive radiochemotherapy, which led to a complete endoscopic response at the end of treatment. The patient's symptom-free survival was six months and overall survival was 17 months from diagnosis. This case of a rare tumor of the upper esophagus raises the question of whether the detection and surveillance of this type of heterotopia would be useful and, thus, to be recommended.
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