When a PanNET Is Not a PanNET: Avoiding the Diagnostic Pitfalls of Peripancreatic Paraganglioma—A Case-Based Review

Paragangliomas are rare extra-adrenal neuroendocrine tumors, with pancreas involvement being extremely uncommon. Their clinical and radiologic similarity with other more common pancreatic neoplasms can cause diagnostic uncertainty. We present a 70-year-old man who presented with acute cholangitis, and a 4.4 cm pancreatic tail mass was found incidentally. Biopsy suggested a well-differentiated neuroendocrine tumor. Despite imaging and biopsy, the diagnosis was made postoperatively with histopathology revealing a paraganglioma in the peripancreatic fat. This case and brief review highlights the limitations of current diagnostic modalities and the need for a comprehensive evaluation in distinguishing paragangliomas from pancreatic neuroendocrine tumors, which is essential for surgery, genetic counseling, and long-term management.