Huge solid pseudopapillary tumor of the pancreas 'Frantz tumor': A case report

Abstract

Solid pseudopapillary neoplasms (SPN) of the pancreas are rare neoplasms accounting for 1-2% of all pancreatic tumors and have a general female predominance. We report a case and intraoperative videos of SPN involving the whole pancreatic tail. A 19-year-old female patient initially presented to another healthcare facility complaining of abdominal pain, which was started 6 years ago. A contrast-enhanced Computed Tomography (CT) scan of the abdomen showed a large mass measuring 15.6 cm × 11.6 cm × 11 cm, arising from the pancreas with an enhancing cystic component. The patient underwent exploratory laparotomy, which revealed a huge mass occupying most of the abdominal cavity. Thus, we proceeded with a distal pancreatectomy and splenectomy. Intraoperatively, the frozen section showed that the mass had features of a solid pseudopapillary tumor of the pancreas with negative resection margins. The SPN diagnosis was confirmed by histopathology and immunohistochemistry. The pathophysiology behind the development of SPN and its cellular origin is still a matter of debate with multiple proposed hypotheses. SPNs are asymptomatic in almost 70% of all cases and usually discovered incidentally. The pre-operative diagnosis of SPNs remains a clinical challenge despite all the current advances in the diagnostic modalities. Surgical management with negative resection margins is the mainstay of treatment, even with metastasis and vascular invasion, surgical excision should be performed whenever feasible. The recurrence rate after surgical resection has been reported to be 3-9%. The prognosis of SPN limited to the pancreas is generally excellent with over 95% cure rate following complete surgical resection. SPN is a rare entity of a controversial origin but is considered as a low-grade malignancy. Surgical resection to achieve complete excision constitutes the mainstay of treatment, which mostly results in an excellent prognosis.