Solid pseudopapillary neoplasms of the pancreas (SPN) have been reported increasingly; however, series focusing on portal-superior mesenteric vein (PV/SMV) or adjacent organ resection are limited in the literature. The aim of this study was to present our experience in treating patients with SPN who underwent this extensive resection. Ten eligible patients were retrospectively reviewed and analyzed. Eight females and two males with a median age of 23 years (range, 11 to 58 years) and a median tumor diameter of 12 cm (range, 4 to 20 cm) were observed. All patients had imaging signs of vascular and/or adjacent organ involvement. Resection with curative intent was performed in all patients; eight underwent synchronous PV/SMV resection and two underwent synchronous left nephrectomy. Malignant SPN was confirmed in seven patients. Postoperative mortality was nil and morbidity occurred in five patients. At a median follow-up of 67.5 months (range, 12 to 110 months), nine patients were alive with no evidence of disease and one died of liver metastases. In conclusion, malignant SPN are low-grade tumors with good prognosis. More aggressive attitude should be adopted when PV/SMVor adjacent organ involvement is indicated on preoperative imaging. En bloc synchronous PV/SMV or adjacent organ resection should be applied, when necessary, to achieve complete resection.
En bloc resection for intra-abdominal/retroperitoneal desmoid-type fibromatosis with adjacent organ involvement: A case series and literature review
