Lymphoplasmacytic sclerosing pancreatitis presenting as a pancreatic head mass in a child: case report and management recommendations

Lymphoplasmacytic sclerosing pancreatitis (LPSP) is an autoimmune form of chronic pancreatitis found most commonly in elderly men and only rarely in children. A 10-year-old boy presented with a 3-week history of obstructive jaundice. Imaging studies showed a pancreatic head mass, hepatic ductal dilatation, and involvement of the portal vein. A preliminary diagnosis of malignancy was based on endoscopic ultrasound characteristics and fine-needle aspiration cytology. The patient underwent a pancreaticoduodenectomy. The patient recovered uneventfully and was discharged home on postoperative day 6. The final pathological diagnosis was LPSP. Lymphoplasmacytic sclerosing pancreatitis is a rare form of chronic pancreatitis in children that is difficult to distinguish from malignancy preoperatively. We discuss the diagnosis and treatment of LPSP. Determination of elevated IgG4 levels in children with pancreatic head masses may allow for the medical treatment of LPSP.