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The tumor typically originates in the skin and exhibits invasive growth into deeper tissues, often manifesting as a firm, poorly defined purplish\u2010red mass. Cases of KHE involving visceral organs, including the bone, retroperitoneum, or mediastinum, have also been reported; however, their clinical features are often nonspecific, which may lead to delayed diagnosis. Importantly, these tumors are frequently associated with Kasabach\u2013Merritt phenomenon, a consumptive coagulopathy that represents a major cause of mortality in KHE. Furthermore, tumor location, size, and clinical response to pharmacotherapy are closely associated with patient prognosis. Therefore, early recognition and timely treatment of KHE are essential. This article aims to review the epidemiology, etiology, clinical manifestations, diagnosis, and management of KHE.<\/jats:p>","DOI":"10.1002\/ped4.70041","type":"journal-article","created":{"date-parts":[[2026,1,31]],"date-time":"2026-01-31T15:52:14Z","timestamp":1769874734000},"page":"269-280","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Kaposiform hemangioendothelioma: Diagnosis and treatment"],"prefix":"10.1002","volume":"10","author":[{"given":"Yi","family":"Tian","sequence":"first","affiliation":[{"name":"Department of Dermatology Beijing Children's Hospital, Capital Medical University, National Center for Children's Health  Beijing China"},{"name":"Key Laboratory of Major Diseases in Children Ministry of Education  Beijing 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