Oesophageal neuroendocrine tumours—case series of a rare malignancy. Issue 12 (20th December 2022)

• Record Type: Journal Article
• Authors: Nikolic, Amanda L; Gullifer, James; Johnson, Mary Ann; Hii, Michael W
• Is Part Of: Journal of surgical case reports. Volume 2022:Issue 12(2022)
• Publication Date: 2022-12-20
• Subjects: case series -- neuroendocrine neoplasm -- oesophageal neoplasm; Surgery -- Periodicals; Surgery -- Case studies -- Periodicals; 617.005
• DOI: 10.1093/jscr/rjac582
• Languages: English
• ISSNs: 2042-8812

Abstract

Abstract: Oesophageal neuroendocrine neoplasms (NENs) are rare tumours. Neuroendocrine carcinomas (NECs) are the highest grade of NENs, with aggressive biological behaviour and poor outcomes. No standardized treatment pathways exist for these tumours, with management being individualized based on patient and tumour factors. We present five cases, four men and one women between 63 and 68 years old, who were diagnosed with symptomatic primary oesophageal NECs. Three were diagnosed with localized disease, and two were diagnosed with metastatic disease. Endoscopy, biopsy and staging scan results are outlined. Two patients received neoadjuvant chemotherapy. Three patients with localized disease underwent oesophagectomy. Two of these patients received neoadjuvant chemotherapy. Four patients have succumbed to their disease, with a median survival following a diagnosis of 18 months (5–34 months). This case series highlights the variability of presentation and stage at diagnosis of oesophageal NECs. Multimodal treatment is commonly utilized; however, outcomes are universally poor. Further research is required to determine the optimal treatment regimen for oesophageal NENS.