小児における破裂性充実性偽乳頭腫瘍：単一施設における20年間の経験

Solid pseudopapillary neoplasm (SPN) is a rare pancreatic tumor with an excellent prognosis after resection. Tumor rupture is uncommon and may increase the risk of peritoneal spread, but data in children are extremely limited. This study evaluated clinicopathologic features, operative outcomes, and recurrence patterns in ruptured SPN in children over 20 years.

We retrospectively reviewed seven children (<18 years) with histologically confirmed ruptured SPN who underwent resection at a tertiary center between 2004 and 2024. Clinicopathologic, operative, and follow-up data were analyzed. Recurrence was categorized as suspected (radiologic) or confirmed (histologic).

All patients were female (median age 12 years). Median tumor size was 7.4 cm, mostly arising from the pancreatic body or tail. Rupture was spontaneous in 42.9% and trauma-related in 57.1%; no iatrogenic rupture events occurred. Distal pancreatectomy was performed in 71.4%. Suspected recurrence occurred in five patients (71.4%), during a median follow-up of 47 months; however, confirmed recurrence was identified in two (28.6%), both with peritoneal dissemination. Both confirmed recurrence cases involved tumors larger than 10 cm with peripancreatic soft tissue extension, although no statistical associations can be inferred due to the small sample size.

Ruptured SPN in children carries a measurable risk of true peritoneal recurrence, but radiologic findings may overestimate relapse. Tumor size and peripancreatic extension may help stratify recurrence risk. Complete resection, thorough peritoneal assessment, and long-term surveillance are essential. Multicenter studies are needed to refine management strategies.