S1960 An Atypical Presentation of Duodenal Neuroendocrine Tumor as Black Esophagus

Abstract

INTRODUCTION: Acute esophageal necrosis (AEN) or “black esophagus” is characterized by diffuse circumferential black esophageal mucosa that abruptly stops at the gastroesophageal (GE) junction. Etiology of AEN is unclear but ischemia, infection, prolonged vomiting, toxin injury, and malignancy are thought to be associated with AEN. We present a patient with vomiting, diarrhea, and weight loss, found to have AEN and a duodenal neuroendocrine tumor (NET) on endoscopy. This case is an atypical presentation of duodenal NET as AEN in the setting of chronic acid exposure. CASE DESCRIPTION/METHODS: A 60-year-old male with past medical history of hypertension and atrial fibrillation presented with vomiting 5 times per day, burning abdominal pain, acute on chronic diarrhea, and 25 pound weight loss. Patient had chronic diarrhea for multiple years and reported having a negative endoscopy and colonoscopy 3 years prior. CT scan of chest, abdomen, and pelvis showed diffuse thickening of esophagus but no mass. An endoscopy revealed severe pan-esophagitis with black ulcerations and necrosis that spared the GE junction. Endoscopy also showed a duodenal mass and numerous duodenal ulcers. Esophageal biopsy showed completely eroded mucosa with granulation tissue, acute/chronic inflammation, and fibrinopurulent surface exudates. Pathology of duodenal mass was consistent with a well-differentiated NET (Ki-67 < 2%). Patient underwent robotic distal gastrectomy and gastrojejunostomy. A follow up endoscopy showed resolution of AEN; however, severe esophageal stenosis had developed and required balloon dilation. DISCUSSION: Although the incidence of AEN is low (0.2%), it is associated with high mortality rate (up to 35%) and complications such as esophageal perforation and stricture. Management is supportive and correcting of the underlying condition. Therefore, it is important to recognize AEN and identify its underlying cause. This patient’s vomiting and injury to the esophagus was secondary to his NET. Duodenal NET accounts for 2-3% of all neuroendocrine tumors and < 2% of all gastrointestinal malignancies in the United States. Although duodenal NETs are often indolent, Zollinger Ellison Syndrome occurs in 10% of patients. Symptomatic patients present with abdominal pain, diarrhea, and heartburn. Complications of severe acid hypersecretion can occur with findings of bleeding (32%), esophageal strictures (2%) and perforation (3%). Five-year overall survival is 60-70%.Figure 1.: Initial endoscopy showing multiple areas of black colored ulcers in the background of severe pan-esophagitis.Figure 2.: Initial endoscopy showing a large frond-like/villous and polypoid lesion with no bleeding in the duodenal bulb.Figure 3.: Follow up endoscopy showing LA Grade D esophagitis and intrinsic severe stenosis but resolution of necrotic lesions.