Repeated Resection for Recurrent Metastatic Solid Pseudopapillary Neoplasm of the Pancreas

Objective: Rare disease Background: Solid pseudopapillary neoplasm (SPN) accounts for 1.0% to 2.0% of all pancreatic neoplasms. SPN generally has good prognosis after surgery; however, 10% to 15% of patients have local recurrence or distant metastasis. There have been a few reports of successful surgical resection of isolated recurrent tumors after radical resection and sporadic reports of multiple metastasectomies. Herein, we present a case of recurrent SPN treated by repeated surgeries. Case Report: A 49-year-old woman was referred to our hospital with jaundice and right upper abdominal pain. Computed tomography (CT) scanning revealed a 73x43-mm heterogeneous mass in the pancreatic head. We performed a pancreatoduodenectomy and diagnosed SPN. The patient was discharged without any complications and was followed up by CT once every 6 to 12 months. Six years later, a 15x15-mm tumor was detected in Couinaud segment VI of the liver. A liver biopsy showed a pathological match to the pancreatic tumor. We performed a partial hepatectomy, and the pathology report confirmed metastatic SPN. At 8 and 10 years after the initial surgery, the patient underwent further partial hepatectomies for confirmed solitary liver metastases of SPN. The Ki-67 index increased for each metastasis identified (initial tumor, 1.88%; 6 years, 7.38%; 8 years, 5.53%; 10 years, 11.22%). No further masses were detected, and the patient survived more than 10 years following surgery. Conclusions: Despite histological transformation to high-grade malignant disease, repeated aggressive surgical resection led to long term survival in our patient with SPN.