Background: Kaposiformhemangioendothelioma (KHE) is a rare locally aggressivevascular tumor that may be associated with severe thrombocytopenia withconsumption coagulopathy (Kasabach-Merritt syndrome) in children and earlyadolescents. It usually presents as a soft tissue mass in the trunk, extremities andretroperitoneum. It is very rare to arise from the head and neck. Radiologically,hemangioendothelioma, including Kaposiformhemangioendothelioma, is seen as ahighly vascularized well-enhancing tumor, but no characteristic findings differentiateKaposiformhemangioendothelioma from other soft-tissue tumors. Treatmentmodalities include surgery, steroids, chemotherapy, interferon and sirolimus.Objectives:This report describes an infant with a large right parapharygealKaposiform hemangioendothelioma confirmed by tissue biopsy totally disappearingwith medical treatment.Design/Method:The patient presented in the neonatal period with progressiverespiratory distress and signs of upper airway obstruction, severe thrombocytopeniaand bilateral subconjunctival hemorrhage. Tracheostomy was required to maintain theairway. Surgical intervention was excluded because of the highly vascular tumor.Vascular embolization was not an option hence there was no large feeding vessel to thetumor. She was treated conservatively with intermittent platelet transfusions, steroidsand propranolol with gradual improvement of platelet counts and respiratorysymptom.Results:Follow up MRIs showed slow regression of the tumor until totaldisappearance over one year period with nomalization of coagulopathy and plateletcount. Figure A and B represent MRI images pre- and post therapy.Conclusion:In conclusion medical treatment with propranolol and steroids hasresulted in airway and platelet count stabilization, obviating the need for operative orother modalities of intervention.

Conference Title

ASPHO Annual Meeting

Conference Country

United States of America

Conference Date

April 24, 2013 - April 27, 2013

Conference Sponsor

American society of Pediatric Hematology Oncology