Middle-Preserving Pancreatectomy for Multicentric Solid Pseudopapillary Neoplasm in a 10-Year-Old Female

• Published in: European journal of pediatric surgery reports 2024-01, Vol.12 (1), p.e58-e62
• Main Authors: Marshall, Grace, Byrne, Matthew, Wirth, Korry, Liao, Xiaoyan, Linehan, David C, Wilson, Nicole A
• Format: Article
• Language: English
• Subjects: Case Report; Frantz tumor; multicentric solid pseudopapillary neoplasm; pancreas; pediatric; urogenital abnormalities
• ISSN: 2194-7619; 2194-7627
• DOI: 10.1055/s-0044-1791812

Solid pseudopapillary neoplasm (SPN) is a rare low-grade malignant tumor of the pancreas that occurs predominantly in young females. This tumor is occasionally multicentric, posing a unique surgical conundrum for resection. We present a case of a 10-year-old female with a history of multicystic dysplastic left kidney and persistent urogenital sinus who was diagnosed with biopsy-proven multicentric SPN of the pancreatic head and tail and underwent middle-preserving pancreatectomy. The patient tolerated the surgery very well. Our case is one of the few reported cases of multicentric SPN in a pediatric patient, and the only case treated with middle-preserving pancreatectomy, which is a novel surgical option for protecting pediatric patients from total endocrine and exocrine pancreatic insufficiency. With the increase in the incidence of SPN, there is an increasing need for pancreas-preserving surgical options, particularly in pediatric patients.