Management of a solid pseudopapillary tumor of the pancreas with liver metastases

Abstract

Solid pseudopapillary tumor, otherwise known as solid and cystic tumor or Frantz tumor, is an unusual form of pancreatic carcinoma. Its natural history differs from the more common pancreatic adenocarcinoma in that it has a female predilection, is more indolent, and carries a better prognosis. Metastatic disease can occur, usually involving the liver, and its management is not well defined. A young woman was found to have a tumor situated in the pancreatic tail with seven synchronous hepatic metastases. Segments II and III were free of metastatic disease. A needle biopsy of the pancreatic lesion was non-diagnostic but suggested that the lesion was unlikely to be a typical adenocarcinoma. Initial treatment consisted of a distal pancreatectomy, which confirmed the diagnosis of a solid pseudopapillary tumor. Given the indolent nature of this pathologic entity as well as the patient's youth, an aggressive approach to treatment of the hepatic metastases was recommended. Because the liver was fatty, the right portal vein was embolized to produce hypertrophy of the left hemiliver. Six weeks later an extended right hepatectomy cleared the hepatic disease. The patient had an uncomplicated recovery, and she remains disease-free at 22 months. Solid pseudopapillary tumor of the pancreas is a rare malignancy. Survival following primary resection approaches 95% at 5 years. Metastatic disease, although rare, usually involves the liver and/or peritoneum. Given the paucity of reported cases, the management of metastatic disease is, to date, poorly defined. This case demonstrates a favorable short-term outcome with aggressive surgical treatment of both the primary and metastatic tumor.