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Pancreatic Neuroendocrine tumours usually manifest as solid tumours. Cystic Pancreatic Neuroendocrine tumours are rare entities, which pose diagnostic challenges. Their clinical and pathological features are not well characterized. Here, we report a cystic Pancreatic Neuroendocrine tumours case, together with literature review, highlighting the features of this tumour. A 62-year-old female presented with vague abdominal symptoms and was subsequently found to have large cystic lesion arising from the head of pancreas, compressing the portal and superior mesenteric vein. She underwent pylorus preserving pancreatico-duodenectomy. Histology confirmed grade III Pancreatic Neuroendocrine tumours. No postoperative complications were observed. It is not yet confirmed whether cystic Pancreatic Neuroendocrine tumours are due to necrosis/degeneration of solid Pancreatic Neuroendocrine tumours or a distinct clinical entity with different characteristics. Irrespective of this discussion, cystic Pancreatic Neuroendocrine tumours shows clinically and pathologically distinct features. Awareness and proper recognition will help in their diagnosis. Resection is the treatment of choice. Further genetic/molecular studies will much more characterize this tumour, thereby broadening the treatment options.
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