Solid pseudopapillary neoplasm of the pancreas with multiple liver metastases successfully managed with staged resection

Abstract

Due to the rarity of the disease, there are no guidelines or consensus for the management of solid pseudopapillary neoplasm of the pancreas with liver metastasis. We describe a patient with solid pseudopapillary neoplasm of the pancreas with multiple liver metastases who was successfully managed with staged resection. A previously healthy 50-year-old female presented to the clinic with dull epigastric pain. The contrast-enhanced computed tomography showed a well-defined cystic lesion at the distal body and tail of the pancreas with multiple liver deposits. A core needle liver biopsy was suspicious of a hepatic epithelioid hemangioendothelioma. A distal pancreatectomy with splenectomy and a left hemi-hepatectomy were performed with a subsequent staged right posterior sectionectomy to address the inadequate future liver remnant. The final histology with immunohistochemistry revealed a pancreatic solid pseudopapillary neoplasm. The patient had satisfactory surgical and oncological outcomes with no recurrence. As these are slow growing tumours, appropriately spaced staged liver resection was feasible to allow hypertrophy of the liver remnant and physiological recovery of the patient. The discrepancy in the histology and cross-sectional imaging led to a pre-operative diagnostic dilemma, and the final diagnosis was reached after surgical resection. Thus, resectable metastatic solid pseudopapillary neoplasm of the pancreas may be offered a primary curative radical surgery before considering other treatment modalities.