Solid Pseudopapillary Tumor of Pancreas with Sinistral Portal Hypertension: A Rare Presentation

An elderly male presented with complaint of dull aching pain in left upper abdomen of 2 months' duration. He was well built, and on abdominal examination, a large lump was palpable in left lumbar region. His biochemical, hematological parameters and tumor markers including CA 19.9 were within prescribed normal limits. Contrast enhanced computed tomography (CT) of the abdomen revealed large, well-defined, mixed solid, and cystic lesion arising from distal body-tail of the pancreas extending up to splenic hilum. Chronic thrombosis involving retro pancreatic splenic vein with multiple perisplenic, peripancreatic, and perigastric collaterals were noted. Based on characteristic CT abdomen findings, a diagnosis of solid pseudopapillary tumor (SPT) of the pancreas with sinistral portal hypertension (SPH) was made. The patient was planned for open distal pancreatectomy with splenectomy. At surgery, splenic flexure of colon was densely adhered to the tumor, and hence en bloc resection of colon was also performed. Postoperative period was uneventful, and he was discharged from the hospital on postoperative day 6. Histopathology reported solid pseudopapillary tumor of the pancreas, 22 cm in the largest dimension. SPT of the pancreas rarely present with SPH. At 22-cm size, this may be one of the largest SPTs reported in the English literature to date.