Management of pancreatic kaposiform hemangioendothelioma with sirolimus in a pediatric patient: a case report and literature review.

Background: Kaposiform hemangioendothelioma (KHE) is a rare locally aggressive vascular tumor. The pancreas is not a common site of KHE, especially in pediatric patients. Given that no guidelines are available for the treatment of KHE, management is currently based on expert opinions and clinical experiences. Here, we report a case of pancreatic KHE with obstructive jaundice, which was treated successfully with oral sirolimus instead of radical surgery. Additionally, a literature review on pancreatic KHE was performed to summarize prior clinical experiences and the available treatments. Case Description: A 10-month-old Chinese male infant presented with obstructive jaundice without any signs of fever, abdominal pain, or distention. A detailed consultation revealed an uneventful history. The obstructive jaundice worsened significantly during 3 weeks of conservative therapy. A pancreatic mass was identified via radiological evidence, and a laparoscopic biopsy of the tumor was performed, which confirmed the diagnosis of pancreatic KHE based on histological findings. Oral sirolimus 0.8 mg/m Conclusions: The pancreas is a rare location of KHE, which is a locally aggressive vascular tumor. Diagnosis is based on histological findings, and therapy should be multidisciplinary and individualized. Although sirolimus has been very successful in the treatment of KHE even without radical surgery, the possible risks of tumor recurrence and adverse effects warrant some caution.