P4016 - A Rare Case of a Mixed Neuroendocrine Carcinoma of the Esophagus

Vikash Chadive, MD¹, Harleen Kaur, MD², Vivek Gumaste, MD^2
1CarePoint Health - Bayonne Medical Center, North Bergen, NJ; ²CarePoint Health - Bayonne Medical Center, Bayonne, NJ

Introduction: Neuroendocrine carcinoma (NEC) of the esophagus is a rare condition accounting for 0.4% to 2% of esophageal malignancies. Mixed neuroendocrine non-neuroendocrine neoplasms (MiNENs) defined as tumors consisting of NEC and either adenocarcinoma or squamous cell carcinoma (SCC) component are even more rare. Here, we present a case of 71-year-old male who presented with dysphagia and weight loss and was found to have a mixed NEC of the esophagus.

Case Description/Methods: 71-year-old male with past medical history of coronary artery disease presented for 3 months of epigastric pain and progressive dysphagia. Patient endorsed 50 pound unintentional weight loss over one year. Pertinent labs included hemoglobin 11, alkaline phosphatase 147, and ferritin 18. Esophagogastroduodenoscopy showed a 4 cm, fungating, and ulcerated mass extending from above the gastroesophageal junction (GEJ) into the cardia. Biopsy revealed a poorly differentiated small cell NEC with focal adenocarcinoma. Neoplastic cells expressed AE1/AE3, cytokeratin 7 (CK7), cytokeratin 20 (CK20), carcinoembryonic antigen (CEA), thyroid transcription factor 1 (TTF-1), and synaptophysin (strong and diffuse) with Ki-67 proliferation index of 90%. Positron emission tomography (PET) scan indicated presence of liver metastasis. Large fluorodeoxyglucose (FDG) avid lymph nodes were also seen in the gastrohepatic ligament region consistent with metastasis. The patient did not want to pursue further treatment and expired approximately 2 months after diagnosis.

Discussion: Twenty to forty percent of esophageal NEC have a non-neuroendocrine component, which is either an adenocarcinoma or SCC component. In a study from MD Anderson, of the 40 patients with NEC, 15 had an adenocarcinoma component while one had a SCC. Studies from Asia however report a predominance of SCC in their series. Most mixed NEC tend to be located in the lower esophagus or the GEJ as was in our case. NEC of the esophagus has a worse prognosis in comparison to esophageal adenocarcinoma and SCC. The median survival is 8.2 months despite therapy. The overall survival tends to be better in patients with non-neuroendocrine component than in patients with pure NEC. Recent studies show some survival benefit with surgery and adjuvant therapy based on tumor staging. Although there is no standard treatment for esophageal NEC, a correct diagnosis is important as the treatment for NEC differs from the treatment of SCC or adenocarcinoma of esophagus.



Disclosures:


Vikash Chadive, MD¹, Harleen Kaur, MD², Vivek Gumaste, MD². P4016 - A Rare Case of a Mixed Neuroendocrine Carcinoma of the Esophagus, ACG 2024 Annual Scientific Meeting Abstracts. Philadelphia, PA: American College of Gastroenterology.