Abstract Background Large cell neuroendocrine carcinoma of the oesophagus (LCNEC) is a rare pathology, and one with a high mortality. Due to the rarity of this condition, there is little evidence regarding optimal treatment. There have been no previous systematic reviews investigating treatment of neuroendocrine carcinomas of the oesophagus. Methods A systematic literature review was conducted identifying relevant literature reporting on cases of large cell neuroendocrine carcinomas of the oesophagus, and where available, the treatment of these cases and their outcomes. The review identified all English-language studies on Pubmed, EMBASE, and Cochrane. Results 3373 abstracts were reviewed to reach a shortlist of 35 papers relevant to the topic. Of these, 77 cases across 16 studies reported on treatment administered and survival outcomes. Of 31 patients with large cell neuroendocrine carcinoma of the oesophagus who underwent oesophagectomy, overall survival ranged from 10.5 months to 96 months. This included patients with disease ranging from stage I to stage III, and patients with or without adjuvant chemotherapy +/− radiotherapy. Conclusion The current evidence to direct the management of large cell neuroendocrine carcinoma of the oesophagus remains scarce due to the rarity of cases. From the existing literature, oesophagectomy appears to be of benefit in the management of these patients. There is no evidence to date to support differentiation in treatment strategy between LCNEC and SCNEC, however wider reporting and further research is needed in order to better understand these subtypes of a rare and aggressive cancer and improve survival outcomes.
Hopping et al. (Fri,) studied this question.