1822 Esophageal Neuroendocrine Tumor: A Rare Case of High-Grade Carcinoma of the Esophagus

Abstract

INTRODUCTION: Neuroendocrine tumors (NET) of the esophagus are extremely uncommon. The rarity of esophageal NETs is thought to be due to an underdeveloped neuroendocrine system in the esophagus. When diagnosed, esophageal NETs are usually found in the distal esophagus where neuroendocrine cells are primarily distributed in associated mucosal glands. CASE DESCRIPTION/METHODS: We describe a unique case of a 62-year-old man diagnosed with an esophageal NET. His PMHx is notable for psoriasis, chronic tobacco use (45 pack years), cirrhosis from heavy alcohol use and illicit drug use including cocaine. He first presented to the hospital with complaints of intermittent episodes of chest pain. He underwent full cardiac workup which was unremarkable. Several months later, he returned to clinic with symptoms of progressive dysphagia to solids. Esophagram revealed a 7cm narrowed segment along the distal esophagus with mucosal irregularity concerning for esophageal neoplasm. A follow up CT abdomen/pelvis showed circumferential wall thickening of the distal esophagus, a 5.2cm necrotic lymph node within the gastrohepatic ligament, as well as a smaller necrotic lymph node at the level of the gastroesophageal junction concerning for malignancy. Follow up EGD revealed a fungating mass in the distal esophagus with biopsies confirming tumor cells positive for MNF116, CD56 and synaptophysin; the overall findings were consistent with high grade neuroendocrine carcinoma of the esophagus. The patient started chemotherapy with Carboplatin and Etoposide; he was subsequently treated with single agent Paclitaxel. The metastatic burden continued to worsen with increased involvement of the liver, lymph nodes and most recently the brain. At his most recent clinic visit, he continues to lose weight and still experiences dysphagia to solids though he is tolerating liquids. He will be starting radiation treatments to the brain and also palliative radiation to the GE junction to help alleviate his dysphagia. DISCUSSION: Prompt diagnosis of esophageal NET is critical as these tumors are known to be rapidly progressive and often have already metastasized by the time of diagnosis. Favorable prognostic factors include no lymphatic metastasis, tumor size of <1.0 cm and histology that reveals well-differentiated tumor cells. For unclear reasons, men are disproportionately more likely to develop NET of the esophagus. The most frequent clinical complaint is dysphagia followed by unintentional weight loss, melena and then typical carcinoid symptoms.