Spontaneous Rupture of Solid Pseudopapillary Neoplasm of the Pancreas: A Case Report and Literature Review.

Introduction: Pancreatic solid pseudopapillary neoplasms (SPNs) are rare tumors, accounting for 1%-3% of all pancreatic tumors, with a predilection for young women. Owing to their often asymptomatic nature, SPNs are typically discovered incidentally. Spontaneous rupture of SPNs is extremely rare, with few reported cases. Herein, we report a case of spontaneous SPN rupture and review the literature on similar cases.

Case presentation: A 17-year-old girl presented with sudden, severe left upper abdominal pain and hemorrhagic shock. Contrast-enhanced computed tomography revealed a 13-cm heterogeneous pancreatic tail tumor with internal extravasation and massive ascites, indicative of intraperitoneal hemorrhage. The patient underwent distal pancreatectomy and splenectomy. Histopathological examination confirmed SPN with no vascular invasion. Immunohistochemistry was positive for β-catenin, CD10, CD56, and synaptophysin, with a low Ki-67 index (1%-2%). The patient had an uneventful recovery and was discharged on postoperative day 13.

Conclusions: Spontaneous SPN rupture is an exceedingly rare occurrence, and its underlying mechanisms remain unclear. Ruptured SPNs may pose a higher risk of recurrence and peritoneal dissemination, necessitating long-term follow-up. Further studies are needed to elucidate the factors influencing SPN rupture and its long-term implications.