RESUMEN
Kaposiform hemangioendothelioma (KHE) is a vascular tumor with poor prognosis. We present a child with progressive disability, extreme pain, and autonomic dysfunction due to a retroperitoneal KHE where radiologic characteristics were essential for diagnosis and monitoring of response to therapy. He received sirolimus, and the symptomatology resolved completely. Repeat MRIs revealed fast marked decrease in vascularity of the tumor, although the volume was not significantly affected. We suggest that the sirolimus-induced tumor de-vascularization may explain the clinical and coagulopathy improvement.
Asunto(s)
Antibióticos Antineoplásicos/uso terapéutico , Hemangioendotelioma/irrigación sanguínea , Hemangioendotelioma/tratamiento farmacológico , Síndrome de Kasabach-Merritt/irrigación sanguínea , Síndrome de Kasabach-Merritt/tratamiento farmacológico , Neoplasias Retroperitoneales/irrigación sanguínea , Neoplasias Retroperitoneales/tratamiento farmacológico , Sarcoma de Kaposi/irrigación sanguínea , Sarcoma de Kaposi/tratamiento farmacológico , Sirolimus/uso terapéutico , Preescolar , Hemangioendotelioma/diagnóstico , Humanos , Síndrome de Kasabach-Merritt/diagnóstico , Masculino , Imagen Multimodal , Neovascularización Patológica , Neoplasias Retroperitoneales/diagnóstico , Sarcoma de Kaposi/diagnósticoAsunto(s)
Embolización Terapéutica , Neoplasias de Cabeza y Cuello/terapia , Recien Nacido Prematuro , Síndrome de Kasabach-Merritt/terapia , Edad Gestacional , Neoplasias de Cabeza y Cuello/irrigación sanguínea , Neoplasias de Cabeza y Cuello/patología , Humanos , Recién Nacido , Síndrome de Kasabach-Merritt/irrigación sanguínea , Síndrome de Kasabach-Merritt/patología , Imagen por Resonancia Magnética , Resultado del Tratamiento , Carga TumoralRESUMEN
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.