Large cell neuroendocrine carcinoma at the esophagogastric junction. An extremely rare entity.

We present the third case described to date of large cell neuroendocrine carcinoma located at the esophagogastric junction (LCNEC). Esophageal neuroendocrine tumours account for 0.03-0.05% of all malignant esophageal tumours. Within oesophageal NETs, LCNEC accounts for 1% of esophageal NETs. This tumour type is characterised by elevated levels of certain markers: synaptophysin, chromogranin A and CD56. In fact, 100% of patients will have chromogranin or synaptophysin, or at least one of these three markers. In turn, 78% will have lymphovascular invasion and 26% will have perineural invasion. Only 11% of patients will have stage I-II, which implies an aggressive course and worse prognosis.