Management of Refractory Pediatric Kaposiform Hemangioendothelioma With Sirolimus and Aspirin. Issue 4 (May 2018)

• Record Type: Journal Article
• Authors: MacFarland, Suzanne P.; Sullivan, Lisa M.; States, Lisa J.; Bailey, L. Charles; Balamuth, Naomi J.; Womer, Richard B.; Olson, Timothy S.
• Is Part Of: Journal of pediatric hematology/oncology. Volume 40:Issue 4(2018)
• Publication Date: 2018-05
• Subjects: hemangioendothelioma -- kaposiform -- sirolimus; Pediatric hematology -- Periodicals; Tumors in children -- Periodicals; 618.9215
• DOI: 10.1097/MPH.0000000000001046
• Languages: English
• ISSNs: 1077-4114

Abstract

Abstract : Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor characterized by aggressive local invasion and a syndrome of platelet trapping known as Kasabach-Merritt phenomenon that, through deposition of platelet derived growth factors, may perpetuate the growth of the tumor. Although many cases of KHE are successfully treated with local control or low-intensity chemotherapy, some cases are often refractory even to aggressive treatment. Herein, we describe a patient with a refractory, recurrent KHE despite multiple attempts at local control and intensive chemotherapy, that ultimately was successfully treated with rationally designed and low-intensity combination therapy of sirolimus and aspirin.