Systematic Review and Pooled Analysis of 1,021 Children With Pancreatic Tumors

Although relatively rare, pediatric pancreatic tumors have been increasingly reported in the literature. Our aim was to systematically review the literature on the clinical characteristics, management, and outcomes of pediatric pancreatic tumors.

We performed a systematic review of the MEDLINE, Scopus, Web of Science, and Cochrane Library databases (end-of-search date: June 2022) according to the Preferred Reporting Items for Systematic Reviews and Meta-analyses statement.

Sixty-four studies reporting in 1021 pediatric patients (< 18 y) were included. The most common tumor histology was solid pseudopapillary neoplasm (SPN) (70.4%), followed by pancreatoblastoma (12.7%), and pancreatic neuroendocrine tumor (pNET) (9.7%). Most children were symptomatic (range: 71.4%-100.0%) and presented with abdominal pain, palpable mass, or nausea and vomiting. The head of the pancreas was the most common location for most tumors (range: 31.3%-70.0%). About 14% of SPNs were characterized as malignant. Surgical resection was performed in 93.5% of patients, and the most common operations were pancreaticoduodenectomy (32.9%), distal pancreatectomy (42.1%), and enucleation (12.2%). Margin-negative resection was achieved in 92.7%. Neoadjuvant chemotherapy was administered in 8.8%. The most common complications observed were fistula in 12.9%, fluid collection in 3.4%, and exocrine insufficiency in 2.7%. The 5-y survival rates were 97.4% for SPNs, 36.7% for pancreatoblastomas, and 75.0% for pNETs. Recurrence was seen in 4.8% of SPNs, 20.5% of pancreatoblastomas, and 4.4% of pNETs.

Among the common pediatric pancreatic tumors, pancreatoblastoma was associated with the worst prognosis and highest recurrence rate. Complete surgical resection remains the mainstay of treatment for pediatric pancreatic tumors with relatively low reported morbidity.