Kaposiform Hemangioendothelioma: Current Knowledge and Future Perspectives

Overview

Journal Orphanet J Rare Dis

Publisher Biomed Central

Specialty General Medicine

Date 2020 Feb 5

PMID 32014025

Citations 61

Authors

Yi Ji

Siyuan Chen

Kaiying Yang

Chunchao Xia

Li Li

Affiliations

Soon will be listed here.

Abstract

Kaposiform hemangioendothelioma (KHE) is a rare vascular neoplasm with high morbidity and mortality. The initiating mechanism during the pathogenesis of KHE has yet to be discovered. The main pathological features of KHE are abnormal angiogenesis and lymphangiogenesis. KHEs are clinically heterogeneous and may develop into a life-threatening thrombocytopenia and consumptive coagulopathy, known as the Kasabach-Merritt phenomenon (KMP). The heterogeneity and the highly frequent occurrence of disease-related comorbidities make the management of KHE challenging. Currently, there are no medications approved by the FDA for the treatment of KHE. Multiple treatment regimens have been used with varying success, and new clinical trials are in progress. In severe patients, multiple agents with variable adjuvant therapies are given in sequence or in combination. Recent studies have demonstrated a satisfactory efficacy of sirolimus, an inhibitor of mammalian target of rapamycin, in the treatment of KHE. Novel targeted treatments based on a better understanding of the pathogenesis of KHE are needed to maximize patient outcomes and quality of life. This review summarizes the epidemiology, etiology, pathophysiology, clinical features, diagnosis and treatments of KHE. Recent new concepts and future perspectives for KHE will also be discussed.

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References

Lohela M, Bry M, Tammela T, Alitalo K . VEGFs and receptors involved in angiogenesis versus lymphangiogenesis. Curr Opin Cell Biol. 2009; 21(2):154-65. DOI: 10.1016/j.ceb.2008.12.012. View

Kim D, Chung J, Ahn S, Kwon T . Laryngeal kaposiform hemangioendothelioma: case report and literature review. Auris Nasus Larynx. 2009; 37(2):258-62. DOI: 10.1016/j.anl.2009.05.003. View

Shirley M, Tang H, Gallione C, Baugher J, Frelin L, Cohen B . Sturge-Weber syndrome and port-wine stains caused by somatic mutation in GNAQ. N Engl J Med. 2013; 368(21):1971-9. PMC: 3749068. DOI: 10.1056/NEJMoa1213507. View

Triana P, Dore M, Nunez Cerezo V, Cervantes M, Sanchez A, Miguel Ferrero M . Sirolimus in the Treatment of Vascular Anomalies. Eur J Pediatr Surg. 2016; 27(1):86-90. DOI: 10.1055/s-0036-1593383. View

Greene A, Goss J . Vascular Anomalies: From a Clinicohistologic to a Genetic Framework. Plast Reconstr Surg. 2018; 141(5):709e-717e. PMC: 5922803. DOI: 10.1097/PRS.0000000000004294. View

Drolet B, Trenor 3rd C, Brandao L, Chiu Y, Chun R, Dasgupta R . Consensus-derived practice standards plan for complicated Kaposiform hemangioendothelioma. J Pediatr. 2013; 163(1):285-91. DOI: 10.1016/j.jpeds.2013.03.080. View

Zhou S, Wang L, Panossian A, Anselmo D, Wu S, Venkatramani R . Refractory Kaposiform Hemangioendothelioma Associated with the Chromosomal Translocation t(13;16)(q14;p13.3). Pediatr Dev Pathol. 2015; 19(5):417-420. DOI: 10.2350/15-09-1707-CR.1. View

Zaldivar M, Pauels K, von Hundelshausen P, Berres M, Schmitz P, Bornemann J . CXC chemokine ligand 4 (Cxcl4) is a platelet-derived mediator of experimental liver fibrosis. Hepatology. 2010; 51(4):1345-53. DOI: 10.1002/hep.23435. View

Verheul H, Jorna A, Hoekman K, Broxterman H, Gebbink M, Pinedo H . Vascular endothelial growth factor-stimulated endothelial cells promote adhesion and activation of platelets. Blood. 2000; 96(13):4216-21. View

10.

Peng S, Yang K, Xu Z, Chen S, Ji Y . Vincristine and sirolimus in the treatment of kaposiform haemangioendothelioma. J Paediatr Child Health. 2019; 55(9):1119-1124. DOI: 10.1111/jpc.14370. View

11.

Fahrtash F, McCahon E, Arbuckle S . Successful treatment of kaposiform hemangioendothelioma and tufted angioma with vincristine. J Pediatr Hematol Oncol. 2010; 32(6):506-10. DOI: 10.1097/MPH.0b013e3181e001a9. View

12.

Iwami D, Shimaoka S, Mochizuki I, Sakuma T . Kaposiform hemangioendothelioma of the mediastinum in a 7-month-old boy: a case report. J Pediatr Surg. 2006; 41(8):1486-8. DOI: 10.1016/j.jpedsurg.2006.04.013. View

13.

Le Cras T, Mobberley-Schuman P, Broering M, Fei L, Trenor 3rd C, Adams D . Angiopoietins as serum biomarkers for lymphatic anomalies. Angiogenesis. 2016; 20(1):163-173. DOI: 10.1007/s10456-016-9537-2. View

14.

Ryu Y, Choi Y, Cheon J, Kim W, Kim I, Park J . Imaging findings of Kaposiform Hemangioendothelioma in children. Eur J Radiol. 2016; 86:198-205. DOI: 10.1016/j.ejrad.2016.11.015. View

15.

Croteau S, Liang M, Kozakewich H, Alomari A, Fishman S, Mulliken J . Kaposiform hemangioendothelioma: atypical features and risks of Kasabach-Merritt phenomenon in 107 referrals. J Pediatr. 2012; 162(1):142-7. PMC: 3494787. DOI: 10.1016/j.jpeds.2012.06.044. View

16.

Knopfel N, Walchli R, Luchsinger I, Theiler M, Weibel L, Schwieger-Briel A . Congenital hemangioma exhibiting postnatal growth. Pediatr Dermatol. 2019; 36(4):548-549. DOI: 10.1111/pde.13813. View

17.

Konczyk D, Goss J, Maclellan R, Greene A . Association between extremity kaposiform hemangioendothelioma and lymphedema. Pediatr Dermatol. 2017; 35(1):e92-e93. DOI: 10.1111/pde.13373. View

18.

Wojtukiewicz M, Sierko E, Hempel D, Tucker S, Honn K . Platelets and cancer angiogenesis nexus. Cancer Metastasis Rev. 2017; 36(2):249-262. PMC: 5557865. DOI: 10.1007/s10555-017-9673-1. View

19.

Adams D, Brandao L, Peterman C, Gupta A, Patel M, Fishman S . Vascular anomaly cases for the pediatric hematologist oncologists-An interdisciplinary review. Pediatr Blood Cancer. 2017; 65(1). DOI: 10.1002/pbc.26716. View

20.

Ji Y, Yang K, Peng S, Chen S, Xiang B, Xu Z . Kaposiform haemangioendothelioma: clinical features, complications and risk factors for Kasabach-Merritt phenomenon. Br J Dermatol. 2018; 179(2):457-463. PMC: 11032113. DOI: 10.1111/bjd.16601. View