Rowan University School of Osteopathic Medicine Stratford, NJ
Matthew Everwine, DO1, Daniel Bodek, DO1, Jeanine M. Chiaffarano, DO2, Donald McMahon, DO3, Brian Blair, DO4; 1Rowan University School of Osteopathic Medicine, Stratford, NJ; 2Jefferson Health New Jersey, Cherry Hill, NJ; 3Rowan School of Osteopathic Medicine/Jefferson University, Turnersville, NJ; 4Jefferson Health, Cherry Hill, NJ
Introduction: Esophageal malignancies are relatively uncommon and arise in approximately 5 out of 100,000 patients. The most encountered types include squamous cell carcinoma and adenocarcinoma. Neuroendocrine tumors of the gastrointestinal (GI) tract are rare, especially within the esophagus, though their presence is rising in recent decades. We document a case of an esophageal adenocarcinoma with neuroendocrine features that presented as right sided abdominal pain.
Methods: The patient was a 71 year old male that presented to the hospital complaining of right side abdominal pain over one week. He denied associated symptoms. On presentation, labs showed a total bilirubin 1.5, direct bilirubin 0.5, Alkaline phosphatase 482, AST 201, and ALT 63. He underwent a CT of the chest and abdomen which showed bilateral pulmonary nodules and multiple liver lesions, the largest within the right hepatic lobe measuring 11.8x10.7cm. Liver biopsy was obtained and demonstrated poorly differentiated carcinoma with neuroendocrine features. Immunohistochemical stain was positive for ae1/ae3 cytokeratin, CKD20, CD56 and Synaptophysin, CDX-2, and p40. The patient subsequently underwent endoscopy and was found to have a 5cm mass at the gastroesophageal junction. Biopsy showed adenocarcinoma with focal neuroendocrine features in the setting of Barrett’s esophagus with high grade dysplasia. Immunohistochemical stain was positive for CK7, CKD20, Chromogranin, Synaptophysin, CDX-2, and rare p40. Discussion: Neuroendocrine tumors account for 2% of malignant tumors within the GI tract. The most frequently affected sites include the small intestine, rectum, appendix, colon, and stomach. The esophagus accounts for 0.04-1.4% of those within the GI tract. Lesions may be mixed, such as neuroendocrine differentiation in adenocarcinoma. These lesions are uncommon in areas outside of the colon and stomach. Mixed lesions often lose neuroendocrine differentiation in invasive adenocarcinoma and are most typically found in low grade dysplasia. The atypia of our patient’s case included high grade dysplasia in his primary disease and metastasis with maintained neuroendocrine differentiation. Given the rarity of this disease, therapeutic regimens have not formally been established. Thus, lung neuroendocrine tumor protocols are frequently instituted to treat esophageal counterparts. Based on these guidelines, our patient is going to receive FOLFOX therapy.
Image 1. Esophageal mass
Image 2. Gastroesophageal junction biopsy showing adenocarcinoma in the background of Barrett's esophagus (100x).
Image 3. Gastroesophageal junction biopsy showing focal positivity for synaptophysin immunostain, demonstrating adenocarcinoma with focal neuroendocrine features (100x).
Disclosures: Matthew Everwine indicated no relevant financial relationships. Daniel Bodek indicated no relevant financial relationships. Jeanine Chiaffarano indicated no relevant financial relationships. Donald McMahon indicated no relevant financial relationships. Brian Blair indicated no relevant financial relationships.