Primary esophageal neuroendocrine carcinoma is a rare, aggressive malignancy lacking evidence-based treatment guidelines. The timing and nature of relapse after successful treatment of locoregional disease are not well characterized. We report a patient lacking risk factors for esophageal cancer who rapidly developed extensive disease recurrence 4 months after achieving complete pathologic response to nonsurgical treatment. Although optimal survival for early stage nonmetastatic disease is achieved by esophagectomy with adjuvant therapy, definitive chemotherapy is also appropriate for late stage nonmetastatic patients. There are presently no protocols for maintenance therapy. We highlight complex treatment considerations for this rare malignancy.
© 2022 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of The American College of Gastroenterology.