S1958 Esophageal Neuroendocrine Tumor: A Rare Cause of Malignant Dysphagia

Abstract

INTRODUCTION: The incidence of gastrointestinal (GI) neuroendocrine tumors (NETs) has risen in recent decades; however, esophageal NETs are exceedingly rare, with an estimated prevalence of 0.04% of all NETs. We present a patient with esophageal NET and highlight the unique features of this rare disease. CASE DESCRIPTION/METHODS: A 58-year-old previously health Caucasian man presented with shortness of breath, hypoxia, and 3-week history of progressive solid food dysphagia. The patient denied weight loss, anemia, or GI bleeding symptoms, and hemoglobin was 14. CT scan revealed 9 cm × 7.6 cm posterior mediastinal mass contiguous with a markedly thickened esophageal wall of 2.5 cm. The mass contacted the heart and the pericardium with a pericardial effusion. Additional findings included pleural effusions, bulky mediastinal lymphadenopathy, lower esophageal mass of 6.4 cm × 5.9 cm, and 9 cm conglomeration of gastrohepatic lymphadenopathy. Positive malignant cytology of suspected GI origin was obtained from the thoracentesis and the pericardiocentesis, both needed for hemodynamic improvement. EGD identified a 20 cm long malignant appearing esophageal stricture extending to the GE junction. Esophageal biopsy revealed high grade NET with positive CAM 5.2, pancytokeratin, synaptophysin, CD 56, CDX2 positive staining pattern consistent with NET of GI origin. The malignant dysphagia was palliated with a 23 mm × 155 cm esophageal stent. The patient is undergoing chemotherapy with carboplatin and etoposide. DISCUSSION: Esophageal NET is an exceedingly rare tumor, estimated prevalence of 1.4% of all GI-NETs, and hence there are few reports about clinical features. Reported findings suggest 80% male predominance, and a majority of patients are either asymptomatic (31%) or present with solid food dysphagia (26.9%). Weight loss (11.5%) and melena (7.7%) were less frequent symptoms in esophageal NETs. The mean tumor size ranged from 0.5 cm to 7 cm. This case illustrates: (1) Weight loss and anemia are not common findings with esophageal NET, when compared to more common esophageal malignancies, (2) A 20-cm long esophageal stricture is a length that has not previously been reported in the medical literature, (3) The extra-esophageal tumor burden without associated weight loss is not typical of common esophageal malignancies. These unusual features should prompt Gastroenterologists to consider rare causes of esophageal malignancies.