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.
Обработка статьи · этапы, ошибки и управление
Ошибки и ограничения
История · было → стало
Состояние обновляется каждые 5 секунд, пока блок открыт. Процент показывается только там, где известен объём работы. Пауза останавливает продолжение этапа; текущий запрос к источнику может завершиться.
Styczewska Malgorzata, Krawczyk Malgorzata A, Gabrych Anna, Kunc Michał, Klosowska Anna, Łyzińska Weronika, Taczanowska-Niemczuk Anna, Skoczen Szymon, Balwierz Walentyna, Rutynowska-Pronicka Olga, Brozyna Agnieszka, Dembowska-Bagińska Bozenna, Latos-Grazynska Elzbieta, Rodziewicz-Konarska Anna, Kazanowska Bernarda, Kalwak Krzysztof, Zielinski Jaroslaw, Walenciak Justyna, Mlynarski Wojciech, Chodała-Grzywacz Agnieszka, Karolczyk Grazyna, Nurzynska-Flak Joanna, Drabko Katarzyna, Plonowski Marcin, Sawicka-Żukowska Małgorzata, Krawczuk-Rybak Maryna, Wawrykow Pawel, Peregud-Pogorzelski Jarosław, Raciborska Anna, Januszkiewicz-Lewandowska Danuta, Skalska-Sadowska Jolanta, Wachowiak Jacek, Derwich Katarzyna, Bereznicka Weronika, Galazka Przemyslaw, Wyrzykowski Dariusz, Przewratil Przemysław, Irga-Jaworska Ninela, Bien Ewa
Аннотация
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.