1829 A Case of Poorly Differentiated Neuroendocrine Neoplasm of the Esophagus

Abstract

INTRODUCTION: Esophageal cancer is a growing public health concern, with its prevalence expected to increase by 140% in the next 10 years. The most common types of esophageal tumors include squamous cell carcinoma and adenocarcinoma, which account for more than 95% of cases, however there are some rarer types of esophageal tumors. We describe a case of poorly differentiated neuroendocrine cancer of the esophagus. CASE DESCRIPTION/METHODS: A 51-year-old Caucasian male with past medical history of hypertension presented with upper abdominal pain for a month. CT abdomen revealed a large 4.5 x 3.5 cm mass in the gastrohepatic region, which prompted endoscopy. He was found to have an ulcerated, cratered 3.5 cm lesion at the mid esophagus on a background of salmon colored mucosa. Pathology showed a poorly-differentiated neuroendocrine carcinoma on a background of Barrett's esophagus, with positive CAM5.2 cytokeratin and CD56 markers and a Ki67 index of 90%. Staging PET-CT showed metastatic lymph nodes in mediastinum and abdomen, as well as metastatic involvement of liver. He was started on systemic chemotherapy with etoposide and cisplatin, and is following with outpatient oncology for further management. DISCUSSION: Neuroendocrine neoplasms (NEN) of the esophagus are very rare, representing only 1% of esophageal malignancies. As in our case, NENs usually occur in older individuals and more commonly in men. Like most esophageal malignancies, they present with dysphagia, abdominal pain, systemic symptoms and at late stages, obstruction; notably, they rarely secrete hormones. On endoscopy, they are usually seen as a subepithelial lesion: a polypoid, nodular elevated lesion with smooth and glistening overlying surface. Diagnosis is made on biopsy, showing the characteristic neuroendocrine cells with abundant neurosecretory granules, which have positive immunohistochemical expression of neuroendocrine markers like synaptophysin and chromogranin. Prognosis depends on the extent of metastasis, determined by cross-sectional imaging and PET-CTs. In general, small superficial NENs < 1 cm can be managed by endoscopic mucosal resection/dissection and NENs >2 cm are treated with surgical resection. For metastatic disease, like in our case, chemotherapy is administered with a combination of platin salts and etoposide. The prognosis for these large tumors remains poor; however rising prevalence of these pathologies warrants a high level of awareness of the condition with treatment options.