Resumo

GUERRERO-PADILLA, Daisy; TANTALEAN-DA FIENO, José; VELASQUEZ-VALDERRAMA, Felipe  e  LEON-PAREDES, Rosa. Thoracic kaposiform hemangioendothelioma with a good response to syrolimus. Case report and literature review. Acta méd. Peru [online]. 2019, vol.36, n.2, pp.124-128. ISSN 1728-5917.

A 4-month girl was admitted to Pediatric Intensive Care Unit (PICU) due to a Kasabach-Merritt Syndrome associated to Kaposiform hemangioendothelioma. After 1-month of treatment with vincristine, steroids, propranolol, and acetylsalicylic acid; her progress was not good, and lesions became larger. Imaging studies (computed tomography and nuclear magnetic resonance) showed involvement of intercostal muscles, pleura, lung parenchyma, and cervical venous thrombosis. Afterwards, the patient developed cyanosis and dyspnea due to an upper airway obstruction, requiring intubation and ventilation support. Sirolimus was started and, on the second week of treatment, both lesion and laboratory parameters improved. The patient was discharged after 42 days of sirolimus therapy. One year later, she is still receiving this drug.

Palavras-chave : Hemangioendothelioma; Neoplasm invasiveness; Sirolimus.

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