Long-term follow-up after pancreatoduodenectomy with portal vein resection for a huge solid pseudopapillary neoplasm in an adolescent girl

A 16-year-old Japanese girl with a huge 13-cm-diameter tumor in the pancreas head presented with life-threatening symptoms and findings including severe anemia, obstructive jaundice, duodenal stenosis, and serious portal vein compression. She underwent a pancreatoduodenectomy with combined resection of the portal vein. Reconstruction of the portal vein was successfully performed using an external iliac vein graft and postoperative anticoagulant therapy. Pathological examination revealed a solid pseudopapillary neoplasm of the pancreas. The patient’s postoperative course was uneventful, but her menstruation ceased for 14 months. She is now alive with no evidence of recurrence 100 months postoperatively and she suffers no impairments in daily activities of life. As a treatment of solid pseudopapillary neoplasms of the pancreas, pancreatoduodenectomy combined with portal vein resection is rarely performed in adolescent patients, but is reportedly successful, with patients tolerating the operation and surviving without recurrence. An aggressive surgical attitude is recommended when dealing with this tumor type with curative resection, even in adolescent patients.