Management of Refractory Pediatric Kaposiform Hemangioendothelioma With Sirolimus and Aspirin.

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.

• Authors: Suzanne P MacFarland, Lisa M Sullivan, Lisa J States, L Charles Bailey, Naomi J Balamuth, Richard B Womer, Timothy S Olson
• Journal: Journal of pediatric hematology/oncology (J Pediatr Hematol Oncol) Vol. 40 Issue 4 Pg. e239-e242 (05 2018) ISSN: 1536-3678 [Electronic] United States
• PMID: 29240034 (Publication Type: Case Reports, Journal Article, Research Support, N.I.H., Extramural)

Chemical References

• Aspirin
• Sirolimus

Topics

• Aspirin (administration & dosage)
• Child
• Hemangioendothelioma (drug therapy, pathology)
• Humans
• Kasabach-Merritt Syndrome (drug therapy, pathology)
• Male
• Sarcoma, Kaposi (drug therapy, pathology)
• Sirolimus (administration & dosage)