Kaposiform hemangioendothelioma: current knowledge and future perspectives

Yi Ji,Chunchao Xia,Li Li,Siyuan Chen,Kaiying Yang

doi:10.1186/s13023-020-1320-1

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.

Highlights

Kaposiform hemangioendothelioma (KHE) is a rare vascular neoplasm that is typically diagnosed in infancy or early childhood
KHE has high morbidity rates, primarily due to local invasive features, compressive effects, or the life-threatening consumptive coagulopathy known as the Kasabach-Merritt phenomenon (KMP) [3,4,5]
We describe the current knowledge and discuss future perspectives on KHE, with

Summary

Introduction

Kaposiform hemangioendothelioma (KHE) is a rare vascular neoplasm that is typically diagnosed in infancy or early childhood. KMP is defined as profound thrombocytopenia, together with consumptive coagulopathy and hypofibrinogenemia associated only with the vascular tumors, KHE and tufted angioma (TA) (Fig. 1) [10, 11]. Rapid enlargement of the lesion with severe KMP development shortly after vaccination has been reported in several patients [18,19,20,21]. Ang-2 levels dramatically decrease with sirolimus treatment in patients with KHE and KMP [44].

Results

Conclusion