Large retroperitoneal paraganglioma concurrent with periampullary adenocarcinoma
An 81-year-old woman with fit physiologic condition, was admitted to our institution in Isfahan, Iran, in 2012, because she experienced progressive abdominal fullness and dull epigastric pain for a few months. Her past medical history was unremarkable. Her blood pressure and heart rate were within normal limits and liver enzymes including serum glutamic oxaloacetic transaminase (SGOT), serum glutamic-pyruvic transaminase (SGPT), alkaline phosphatase and gamma glutamyl transferase showed increased values. Bilirubin level, Carcinoembryonic antigen and cancer antigen 19-9 levels were within normal limits.\nIn the abdominal examination a large mass on upper paramedian, left side was touched. A computed tomography (CT) scan of the abdomen demonstrated a large retroperitoneal solid-cystic mass and significant dilation of gallbladder, intra and extra hepatic and pancreatic ducts [].\nMagnetic resonance cholangiopancreatography of the patient showed large solid-cystic tumor anterior to left psoas muscle and left kidney, inferior to pancreatic tail. It showed either, complete obstruction of main distal pancreatic duct and distal Common Bile Duct (CBD) [].\nThe patient underwent endoscopic retrograde cholangiopancreatography (ERCP), which revealed a tumoral papilla (2 cm × 2 cm × 1 cm) and biopsy was taken. Microscopic examination of papi biopsy showed adenocarcinoma.\nShe was planned for surgery with frozen section examination of abdominal mass. At the time of surgery, an encapsulated, hypervascular mass (approximately 13 cm × 10 cm × 4 cm) was observed in her retroperitoneal area with the central degeneration and hemorrhage and was subsequently excised [].\nPathological analysis of the mass on frozen section showed a cellular lesion with probability of malignancy, without any sign of metastatic carcinoma.\nPancreaticoduodenectomy was considered for management of her periampullary mass. In permanent histologic sections, the abdominal mass revealed an encapsulated tumor; the tumor cells were arranged in sheets, large nests and organoid patterns, and contained mild pleomorphic nuclei with low mitotic activity []. The neoplasm was histologically benign, with no evidence of invasion, compatible with a paragangliom. The histologic evaluation of peiampullary tumor revealed an adenocarcinoma in stage IIB. After three months from patient's surgery, her treatment has been completed and patient seems to be healthy.