Gastric neuroendocrine carcinoma arising from heterotopic pancreatic tissue

An 85 year old man presented with symptoms of dyspepsia and increase in stool frequency of two months duration. Upper endoscopy revealed an ulcer and the biopsy was interpreted as carcinoma with endocrine features. A formal distal gastrectomy was planned, but intraoperatively, because of the patient's condition, a wedge resection was performed. Histology revealed a neuroendocrine tumour (grade 1), located mainly in the submucosa, which had caused mucosal attenuation and ulceration. Associated with the tumour and at its periphery was heterotopic pancreatic tissue composed of acini, ducts, and endocrine cells. The tumour was strongly positive for chromogranin and focally for synaptophysin. There was no associated chronic atrophic gastritis or G cell hyperplasia. A discrete focus of high grade neuroendocrine carcinoma (grade 3) within the typical (grade 1) neuroendocrine tumour was also present. This case illustrates a grade 1 neuroendocrine carcinoma, with a small grade 3 focus, arising within or intimately associated with heterotopic pancreatic tissue in the stomach. Although the two entities may be separate, their close topographical association favours the possibility of neuroendocrine carcinoma arising from the heterotopic pancreas. Pathologists should be aware of the occurrence of pancreatic heterotopia in the stomach and the association of carcinoma with it.