Retrospective Multicenter Study on Childhood Kaposiform Hemangioendothelioma (2007-2024): Report of the Section of Childhood Vascular Anomalies of the Polish Society of Pediatric Oncology and Hematology.
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BACKGROUND: Kaposiform hemangioendothelioma (KHE) is a rare, locally aggressive vascular tumor, typically affecting infants. Kasabach-Merritt phenomenon (KMP) is a frequent and serious complication of KHE. The study aimed to analyze clinical manifestations, treatment strategies, and outcomes in children with KHE treated in Poland between 2007 and 2024. PROCEDURE: Clinical data of 42 children with KHE treated in 15 Polish pediatric hematology/oncology and surgery centers between 2007 and 2024 were analyzed retrospectively. RESULTS: KMP was present in 27/42 children (64.3%). The median age at diagnosis in children with and without KMP was 2.5 and 8 months, respectively. A male predilection was observed (61.9%), particularly in patients without KMP (13/15, 86.7%). Diagnosis of KHE required a tumor biopsy in 20 patients, including 14/15 (93.3%) of patients without KMP. Treatment strategies varied significantly between patients, institutions, and treatment periods. Systemic treatment was administered in 39 (92.9%) children, with predominating role of chemotherapy and glucocorticoids in the first period (2007-2013), and with gradually increasing importance of sirolimus in the years 2014-2018 and 2019-2024. Three children were successfully treated with surgery only. Salvage therapies ultimately controlled KHE progression/relapse in 14/17 (82.4%) patients. Only one child with metastatic, treatment-resistant cardiac KHE died of disease progression. CONCLUSIONS: Management of childhood KHE is challenging since no unified treatment recommendations exist. To optimize KHE therapy in Poland, the Section of Childhood Vascular Anomalies was established in June 2021 as part of the Polish Society of Pediatric Oncology and Hematology. Its aim is to standardize therapeutic guidelines and provide education on vascular anomalies in Poland.