1843 A Rare Case of Large Cell Neuroendocrine Carcinoma of the Esophagus

Abstract

INTRODUCTION: Neuroendocrine Tumors (NETs) are a rare form of esophageal malignancy with an incidence ranging from 0.4 and 2% of all malignant esophageal tumors (1). Among esophageal NETs, the vast majority are small cell esophageal carcinomas. A recent literature review found only 35 cases of large cell esophageal carcinoma have been reported since 1952 (2). CASE DESCRIPTION/METHODS: Patient is a 48-year-old male with past medical history of HTN, diabetes, and HLD who presented with 6 weeks of progressive dysphagia predominantly to solids. The dysphagia was initially infrequent, but eventually occurred every time he ate. ROS was pertinent for a 20 lb weight loss. Social history was pertinent for 1-2 drinks of alcohol per month and daily use of chewing tobacco. Family history revealed a mother with a brain mass and a grandmother with pancreatic CA. PE was normal. He underwent an EGD which showed a large, ulcerative distal esophageal mass extending into gastric cardia. Biopsies revealed a poorly differentiated carcinoma most compatible with large cell neuroendocrine carcinoma. CT imaging of chest, abdomen, and pelvis showed nonspecific lower esophageal mural thickening with no evidence of esophageal mass. PET scan showed hypermetabolic distal esophageal mass measuring 6 x 5 x 3 cm with peri gastric lymph node involvement. The patient underwent neoadjuvant treatment with radiation, cisplatin and etoposide followed by a distal esophagectomy with gastric pull-through. Surgical path revealed Stage III large cell neuroendocrine carcinoma involving the distal esophagus, proximal stomach, and peri gastric lymph nodes. Patient is currently recovering from the surgery with plans for postoperative chemotherapy. DISCUSSION: This case illustrates an rare form of a malignant esophageal neuroendocrine tumor. Esophageal NETs tend to be more aggressive than NETs found in other parts of the GI tract, so early diagnosis and treatment is critical in order to prolong survival. Due to a paucity of cases in the literature, there is a need for more cases to be presented in order to better understand the course of the disease and determine the best modalities for treatment.