Pancreatic hemangioendothelioma, an extremely rare vascular anomaly in children: A case report and literature review

The current study presents the case of a female infant with pancreatic hemangioendothelioma (HE) and discusses this rare disease in terms of the clinical features, treatment principle and prognosis. An 8-month-old female with pancreatic HE was admitted to the Department of General Surgery in The Children's Hospital (Zhejiang University School of Medicine, Hangzhou, Zhejiang, China) on January 3, 2011, due to yellow skin and sclera that had been apparent for 1 week. Magnetic resonance imaging (MRI) showed dilatation in the left and right hepatic, common hepatic and common bile ducts, a pancreatic head mass of 4.7x5.2 cm with a strong signal, and close linkage of the portal vein and mesenteric vessels to the mass. Laparotomy was performed on January 11, 2011. An intraoperative frozen specimen showed a benign tumor. Finally, Whipple surgery retaining the tumor was chosen. The patient was treated by fasting, gastrointestinal decompression, antibiotics and supportive treatment post-surgery. Jaundice disappeared rapidly following the surgery. The patient started eating at 1 week post-surgery and was discharged 1 week later. From the pathological results, a diagnosis of pancreatic HE was made, with no tumor invasion of the hepatic lymph nodes. During the follow-up visit at 28 months post-surgery, the child showed good growth and development. MRI showed that the mass size was 2.4x2.0x1.5 cm, which was a significantly decrease from previously. Additionally, a literature search showed that from 1973 to the present date, only 9 studies on children with HE of the pancreas have been reported. Childhood pancreatic HE is a rare form of pancreatic vascular anomaly, mainly occurring in infants. If the tumor oppresses the duodenum and invades the mesenteric vessels, Whipple surgery retaining the tumor could be performed.