Clinical and imaging features of Kaposiform Hemangioendothelioma

Abstract

Kaposiform hemangioendothelioma (KHE) is a unique locally aggressive vascular tumor with poor prognosis. The aim of this study is to assess the clinical and imaging features of KHE, and to compare the differences between solitary and diffusive infiltrative subtype further. The clinical and radiological findings of a cohort of 25 cases with histologically proven KHE, between June 2011 and June 2016, were reviewed retrospectively. 7 solitary and 18 diffusive infiltrative subtypes KHE were included. The differences of clinical and imaging features between these two subtypes were compared statistically by Wilcoxon rank sum test and Fisher exact test. The median age was 4 months old. 20 cases (80%) were accompanied by Kasabach-Merritt phenomenon (KMP). Most KHE located in trunk and/or extremity. The masses showed inhomogeneous echogenicity and were rich in vascularity on ultrasound; showed isoattenuation relative to muscle on unenhanced CT, isointense(n=15) or slightly hyperintense (n=7) T1 weighted imaging (T1WI) signal relative to muscle, mainly heterogeneous hyperintense or slightly hyperintense with speckled hypointense (n=17) T2WI signal (77%) relative to muscle, and notable (n=15) and moderate (n=3) enhancement. Feeding and draining vessels were revealed in 15 cases. Five masses with DWI showed slightly restricted diffusivity, with average apparent diffusion coefficient value of (1.28±0.09) ×10-3mm2s-1. Necrosis and hemorrhage were also found. Compared with solitary ones, diffusive infiltrative KHE were larger, more commonly accompanied by KMP and reticular lymphedema, and more frequently located in trunk and/or extremity. Five masses with DWI showed slightly restricted diffusivity. A hypervascular mass accompanied by KMP and reticular lymphedema, with speckled hypointense signal T2WI signal, especially in pediatric patients, is highly suggestive of the diagnosis of KHE. Advances in knowledge: Speckled hypointense signal T2WI signal, and notable enhancement were unique features of KHE. KHE showed slightly restricted diffusivity on DWI, commonly accompanied by KMP and reticular lymphedema.