Peripancreatic High-Grade Neuroendocrine Neoplasm Mimicking a Pancreatic Mass in Esophageal Small Cell Carcinoma

Abstract

ABSTRACT Primary small cell carcinoma of the esophagus (SCCE) is an infrequent aggressive neuroendocrine malignancy. We describe a 60-year-old woman with cirrhosis who presented with weight loss, backache, and night sweats. Histopathology confirmed small cell carcinoma of the esophagus with synaptophysin positivity and Ki-67 ∼90%. Endoscopic ultrasound showed a 38-mm lesion peripancreatically. Fine-needle aspiration cytology showed a high-grade neuroendocrine tumor (Ki-67 >50%) with positivity for synaptophysin, AE1/AE3, and insulinoma-associated protein 1, with a lack of tissue adequacy for classification. There was no evidence of pancreatic mass on MRI, thus suggesting metastatic lymphadenopathy. TP53 and RAD50 mutations were identified. Liver dysfunction, thrombocytopenia limited treatment, and the patient died within 6 weeks.