Esophageal adenocarcinomas in heterotopic gastric mucosa: review and report of a case with complete response to neoadjuvant radiochemotherapy

Abstract

Adenocarcinomas are exceedingly rare in the cervical esophagus (26 reported cases), where squamous cell cancer (SCC) is the predominant tumor type. Esophageal heterotopic gastric mucosa (HGM) - a frequent remnant of incomplete replacement of the original columnar epithelium during the embryonic period - is suspected as cellular origin of cervical esophageal adenocarcinomas. As in any rare tumor entity, no standard treatment strategy is available for cervical esophageal adenocarcinomas. We herein report about the case of a 52-year-old man with a locally advanced, irresectable cervical esophageal adenocarcinoma originating in HGM. We decided on a neoadjuvant therapy (48.6 Gy + 5-FU/cisplatin) derived from experiences with SCC. Restaging showed an extraordinary good clinical response of the previously irresectable tumor. Subsequently the patient underwent limited cervical esophageal resection, lymphadenectomy and interposition of a free jejunal loop for reconstruction. Postoperative histopathological work-up of the specimen showed no residual tumor tissue, but unchanged HGM. This is the first case with complete response of a rare cervical esophageal adenocarcinoma to a neoadjuvant protocol. On 3-year follow-up the patient is doing fine with no complaints of dysphagia and no evidence of local or systemic recurrence.