S4354 A Case of Signet-Cell Adenocarcinoma of the Esophagus

Introduction: Esophageal Signet ring cell carcinoma (SRCC) accounts for only 2.6%–5.0% of all esophageal cancers. SRCC are usually featured by poor differentiation, rapid growth, diffuse infiltration and high metastatic rate. The presence of signet-ring cells conferred a worse prognosis, and these tumors responded differently to conventional treatments as compared with typical adenocarcinoma. We report a case of a 77-year-old old man diagnosed with signet ring cell carcinoma of the esophagus with history of chronic Barrett’s esophagus. Case Description/Methods: A 77-year-old man with a history of Barrett’s esophagus under routine surveillance presented for a scheduled repeat endoscopy. His past medical history included chronic gastroesophageal reflux disease, asthma, type 2 diabetes mellitus, hypertension, and benign prostatic hyperplasia. Two years prior, an esophagogastroduodenoscopy revealed a hiatal hernia and biopsy-confirmed Barrett’s esophagus, for which he was initiated on proton pump inhibitor therapy and advised follow-up surveillance. At follow-up, the patient reported no new or worsening gastrointestinal symptoms. Endoscopy was done and biopsies were taken from the gastro-esophageal junction which showed a poorly differentiated adenocarcinoma with numerous signet ring cells highlighted by the PAS/Alcian blue stain and a background esophageal junction/fundic mucosa showing areas of intestinal metaplasia. In view of the biopsy results, computed tomography chest, abdomen and pelvis were done to check for metastasis and their results were negative. The patient is currently being considered for esophagectomy due to the poor prognosis associated with SRCC. Discussion: Esophageal SRCC is a rare aggressive malignancy with a poor prognosis. SRCC is associated with a median range of survival of 10-20 months. The 5-year survival rate drops to 0% in patients having ≥50% signet ring cells on biopsy. It behaves differently from typical adenocarcinoma in response to treatment and are usually diagnosed very late into the disease course. Studies suggest that SRCC’s have a lower rate of complete pathologic response in patients treated with preoperative chemoradiation and surgery. They tend to have more frequent positive margins after resection as compared to other histological types. Recent Nomogram models currently aid in individual risk stratification and prognosis prediction. Further studies are needed to establish evidence-based guidelines for managing this uncommon entity.