PS02.175: CLINICAL CHARACTERISTICS AND TREATMENT OF NEUROENDOCRINE CARCINOMA OF THE ESOPHAGUS
Abstract Background Neuroendocrine carcinoma (NEC) of the esophagus is a rare and highly aggressive disease; however, an appropriate treatment strategy remains to be established, especially for surgical resection. The objective of this study is to clarify the clinical characteristics of NEC of the esophagus and to determine the optimal surgical strategy. Methods Nineteen patients who were immunohistochemically diagnosed with NEC of the esophagus from 1998 to 2017 were included in this study. The clinical features and therapeutic outcomes were examined. Results 1. Clinical features Sixteen of 19 patients showed protruding or localized type with or without ulceration. Only five patients were negative for both lymph node and organ metastasis and eight cases were positive for metastasis to distant organs and/or distant lymph nodes. 2. Surgical treatment Five patients underwent esophagectomy. Four cases were classified as cStage I (cT1bN0M0); the other case was classified as cStage III (cT3N2M0). The preoperative diagnoses, based on the examination of biopsy specimens, were NEC (n = 2), SCC (n = 1), adenocarcinoma (n = 1), and carcinoma (n = 1). Subtotal and distal esophagostomy were performed in 3 and 2 patients, respectively. Salvage esophagectomy for recurrent disease was performed after definitive chemotherapy in one patient. Anastomotic leakage, which was conservatively healed, developed in one patient. Two patients with histologically-positive node metastasis died at 8 and 13 months, respectively, while another 3 patients (pT1bN0, n = 2; pT2N0, patient, n = 1) are currently alive without any recurrence, at 12, 45 and 72 months after esophagectomy, respectively. 3. Non-surgical treatment Systemic chemotherapy was performed as the main treatment for 13 patients with advanced NEC. The regimens included cisplatin combined with irinotecan or etoposide (n = 10), cisplatin plus 5-fluorouracil (n = 2) and UFT plus irinotecan (n = 1). The MST was 13 months (range, 6–17 months). Conclusion The possibility of NEC should be kept in mind when we encounter protruded tumors of a distinctive shape. Consistent with previous reports, the prognosis of NEC of the esophagus is dismal, irrespective of the administration of systemic chemotherapy. However, surgical resection is considered to be a treatment option for cStage I/II NEC without node metastasis, as a long-term survival after esophagectomy was achieved by some patients. Disclosure All authors have declared no conflicts of interest.