Kaposiform hemangioendotheliomas and tufted angiomas: Sirolimus for treating children
- Authors: Kondrashova Z.A.1,2, Donyush E.K.1,2, Korotkaya E.A.2, Kletskaya I.S.1, Garbuzov R.V.1, Polyaev Y.A.1, Myl’nikov A.A.1
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Affiliations:
- Russian Children's Clinical Hospital of Pirogov Russian National Research Medical University
- Pirogov Russian National Research Medical University
- Issue: Vol 27, No 4 (2023)
- Pages: 261-271
- Section: ORIGINAL STUDY
- Submitted: 17.11.2022
- Accepted: 22.10.2023
- Published: 24.10.2023
- URL: https://jps-nmp.ru/jour/article/view/747
- DOI: https://doi.org/10.55308/1560-9510-2023-27-4-261-271
- ID: 747
Cite item
Abstract
Introduction. Kaposiform hemangioendothelioma (KHE) and tufted angioma (TA) are extremely rare vascular tumors characterized by pathologically progressive angiogenesis and lymphangiogenesis. They are also associated with the development of the KazabachMerritt Phenomenon (KMP; Kasabach-Merritt Phenomenon).
Material and methods. 8 patients (5 boys, 3 girls) with kaposiform hemangioendothelioma (two out of them with Kazabakh-Merritt syndrome) and 4 patients (3 boys, 1 girl) with tufted angioma were treated at the Russian Children's Clinical Hospital of Pirogov Russian National Research Medical University (Moscow, Russia) . All patients were prescribed Sirolimus therapy for antiproliferative purpose.
Results. In all children, positive changes were registered: reduction of vascular tumor volume, gradual improvement of clinical picture and control of complications: pain syndrome, KMP, improvement or restoration of functions in affected organs /systems (increase/restoration of movement volume in joints, compensation for length discrepancy in lower legs). Complete resorption of vascular tumors and clinical symptoms were registered in 3 patients.
Conclusion. The obtained encouraging results after Sirolimus therapy prescribed for treating KME and TA allow the authors to recommend this therapy to patients with KME with or without KMP and TA. However, further researches with a large sample of participants is required to confirm the obtained results.
About the authors
Z. A. Kondrashova
Russian Children's Clinical Hospital of Pirogov Russian National Research Medical University;Pirogov Russian National Research Medical University
Author for correspondence.
Email: z.a.kondrashova@gmail.com
ORCID iD: 0000-0001-6382-8057
Zinaida А. Kondrashova, MD, hematologist, department of hematology and chemiotherapy
117997 Moscow
117997, Moscow
РоссияE. K. Donyush
Russian Children's Clinical Hospital of Pirogov Russian National Research Medical University;Pirogov Russian National Research Medical University
Email: fake@neicon.ru
ORCID iD: 0000-0002-4252-8829
117997 Moscow
117997, Moscow
РоссияE. A. Korotkaya
Pirogov Russian National Research Medical University
Email: fake@neicon.ru
ORCID iD: 0000-0003-3196-901X
117997, Moscow
РоссияI. S. Kletskaya
Russian Children's Clinical Hospital of Pirogov Russian National Research Medical University
Email: fake@neicon.ru
ORCID iD: 0000-0002-8552-7682
117997 Moscow
РоссияR. V. Garbuzov
Russian Children's Clinical Hospital of Pirogov Russian National Research Medical University
Email: fake@neicon.ru
ORCID iD: 0000-0002-5287-7889
117997 Moscow
РоссияYu. A. Polyaev
Russian Children's Clinical Hospital of Pirogov Russian National Research Medical University
Email: fake@neicon.ru
ORCID iD: 0000-0002-9554-6414
117997 Moscow
РоссияA. A. Myl’nikov
Russian Children's Clinical Hospital of Pirogov Russian National Research Medical University
Email: fake@neicon.ru
ORCID iD: 0000-0003-3317-3058
117997 Moscow
РоссияReferences
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