Ruptured solid pseudopapillary neoplasm in children: a 20-year single-center experience.

Area of Science:

• Pediatric oncology
• Surgical pathology
• Abdominal imaging

Background:

• Solid pseudopapapillary neoplasm (SPN) is a rare pancreatic tumor with a generally favorable prognosis after surgical resection.
• Tumor rupture in SPN is uncommon but may be associated with an increased risk of peritoneal spread.
• Data on ruptured SPN specifically in the pediatric population are limited.

Purpose of the Study:

• To evaluate the clinicopathologic features, operative outcomes, and recurrence patterns of ruptured SPN in children.
• To assess the risk of peritoneal spread and recurrence following resection of ruptured SPN in pediatric patients.

Main Methods:

• Retrospective review of seven pediatric patients (<18 years) with histologically confirmed ruptured SPN who underwent resection.
• Analysis of clinicopathologic, operative, and follow-up data, including categorization of recurrence as suspected (radiologic) or confirmed (histologic).

Main Results:

• All seven patients were female, with a median age of 12 years and median tumor size of 7.4 cm.
• Rupture was spontaneous in 42.9% and trauma-related in 57.1%. Distal pancreatectomy was the most common procedure (71.4%).
• Suspected recurrence occurred in 71.4% of patients, but confirmed recurrence with peritoneal dissemination was observed in 28.6%, associated with larger tumors (>10 cm) and peripancreatic extension.

Conclusions:

• Ruptured SPN in children presents a risk of true peritoneal recurrence, with imaging potentially overestimating relapse.
• Tumor size and peripancreatic extension may be indicators for stratifying recurrence risk in pediatric ruptured SPN.
• Complete surgical resection, comprehensive peritoneal evaluation, and long-term patient surveillance are crucial for managing this condition.