1741 Large Cell Neuroendocrine Carcinoma of the Esophagus Arising From Barrett’s Esophagus: A Rare Phenomenon

• Published in: The American journal of gastroenterology Vol. 114; no. 1; p. S976
• Main Authors: Shah, Maulik, Truscello, David, Lal, Kailash, Kolnik, John
• Format: Journal Article
• Language: English
• Published: 01.10.2019
• ISSN: 0002-9270; 1572-0241
• DOI: 10.14309/01.ajg.0000596496.41830.38

INTRODUCTION: In this case report, we present an anomalous case of poorly differentiated large cell neuroendocrine carcinoma (NEC) arising in Barrett’s esophagus (BE). BE typically predisposes a patient to an adenocarcinoma of the gastroesophageal junction, although the incidence is low. Proximally, squamous cell carcinomas are a more likely finding in the esophagus. Esophageal large-cell NECs are rare and aggressive tumors and lead to a poor prognosis. CASE DESCRIPTION/METHODS: 56-year-old male presented to the outpatient gastroenterology office with a history of gastroesophageal reflux disease (GERD) and unintentional 22 lb weight loss. He endorsed acid reflux refractory to long-term PPI therapy. Initial upper endoscopy revealed short segment Barrett’s esophagus and gastritis. Follow-up surveillance endoscopy performed six months later demonstrated a cratered ulcer within the segment of Barrett’s esophagus (Figure 1). Biopsies revealed large-cell neuroendocrine carcinoma in the setting of Barrett’s esophagus (Figure 2). Repeat biopsies taken during endoscopic ultrasound confirmed this. The biopsy stained positive for CDX2, CK7, CK20, chromogranin and synaptophysin. Following diagnosis, he was seen by Thoracic Surgery and had Ivor Lewis esophagectomy (ILE) performed. Given its aggressive biology and risk of recurrence, he was evaluated by oncology and started on carboplatin and etoposide chemotherapy treatment. DISCUSSION: Our patient is a middle-aged Caucasian male with a history of GERD, which are known risk factors for development of BE. BE is a precursor for esophageal adenocarcinoma (EAC), with the annual risk of transformation being 0.1-0.5%. Considering BE increases the risk of developing esophageal cancer, this case reminds gastroenterologists of the importance of surveillance endoscopy and biopsy. Although there are a few cases of esophageal large-cell NEC noted in literature, we found no others reporting this subtype in the setting of BE. Neuroendocrine collision tumors in the setting of BE have only been reported in one other case. Esophageal large-cell NEC are extremely rare, accounting for only 0.04-1.4% of GI NECs reported. Given the rarity of esophageal large-cell NECs, there are no definitive guidelines for diagnosis, staging and treatment. Surgery and platinum-based adjuvant chemotherapy have been the only effective treatments. Aggressive biology and high risk of recurrence means it is imperative that an accurate diagnosis is made and prompt treatment is initiated.