Large retroperitoneal paraganglioma concurrent with periampullary adenocarcinoma

Paragangliomas are tumors that originate from extra-adrenal medullary neural crest derivatives. They are rarely located in retroperitoneal space. These tumors are often discovered incidentally during imaging studies performed for other reasons.Periampullary cancers include adenocarcinomas arising from the pancreas, ampulla of Vater, duodenum or distal common bile duct. The exact site of origin of periampullary tumors is often difficult to ascertain pre-operatively. We report the case of a patient who had a retroperitoneal non-functional paraganglioma, concurrent with periampullary adenocarcinoma. An 81-year-old woman was admitted with progressive abdominal fullness. There was an upper paramedian, left sided, large, palpable mass on the physical examination.Laboratory investigations showed an increase in liver enzyme levels. On abdominal computed tomography the patient found to have a large retroperitoneal mass and dilation in biliary tract, which was confirmed by magnetic resonance cholangiopancreatography. She had a tumoral papi in Endoscopic Retrograde cholangiopancreatography. Which biopsy revealed adenocarcinoma. She underwent surgery for excision of abdominal mass and pancreaticoduodenectomy. And pathologic study showed paraganglioma. This is the first ever reported case of concurrent paraganglioma and periampullary adenocarcinoma.