Kaposiform Hemangioendothelioma with Kasabach-Merritt Phenomenon: Successful Treatment with Embolization and Vincristine in Two Newborns

• Published in: Journal of vascular and interventional radiology Vol. 23; no. 3; pp. 417 - 422
• Main Authors: Garcia-Monaco, Ricardo, MD, PhD, Giachetti, Ana, MD, Peralta, Oscar, MD, Napoli, Noelia, MD, Lobos, Pablo, MD, Gioseffi, Laura, MD, Mariani, Gonzalo, MD
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01.03.2012
• Subjects: Antineoplastic Agents, Phytogenic - administration & dosage; Biopsy; Blood Coagulation; Blood Coagulation Tests; Chemotherapy, Adjuvant; Embolization, Therapeutic; Female; Hemangioendothelioma - blood; Hemangioendothelioma - blood supply; Hemangioendothelioma - diagnosis; Hemangioendothelioma - drug therapy; Humans; Infant, Newborn; Kasabach-Merritt Syndrome - blood; Kasabach-Merritt Syndrome - blood supply; Kasabach-Merritt Syndrome - diagnosis; Kasabach-Merritt Syndrome - drug therapy; Platelet Count; Pulse Therapy, Drug; Radiology; Sarcoma, Kaposi - blood; Sarcoma, Kaposi - blood supply; Sarcoma, Kaposi - diagnosis; Sarcoma, Kaposi - drug therapy; Treatment Outcome; Tumor Burden - drug effects; Vincristine - administration & dosage; KMS; APTT; PT; activated partial thromboplastin time; prothrombin time; KHE; kaposiform hemangioendothelioma; Kasabach-Merritt syndrome
• ISSN: 1051-0443; 1535-7732
• DOI: 10.1016/j.jvir.2011.12.007

Abstract Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor and has a high mortality in newborns when associated with Kasabach-Merritt syndrome (KMS). In two newborns with KHE and severe KMS refractory to medical treatment, emergency embolization led to clinical improvement in the acute neonatal setting by reducing tumor volume, increasing the platelet count, and improving other clotting parameters. Systemic vincristine treatment was added for further tumor control. Both patients remained symptom-free at long-term follow-up.