Kaposiform haemangioendothelioma: magnetic resonance imaging features in 64 cases

Background Kaposiform haemangioendothelioma (KHE) is a rare, locally aggressive disorder. The presenting and imaging features of KHE can overlap with other vascular anomalies and tumours. We aimed to analyse the imaging findings of KHE disorder and highlight features most suggestive of this diagnosis. Methods The clinical features and imaging findings were retrospectively reviewed in 64 patients with pathological diagnosis of KHE. Results Of the 64 patients diagnosed with KHE, 36 patients were < 6 months and 28 patients were >= 6 months. The most common presenting features were Kasabach-Merritt phenomenon (KMP, 42.2 %), visible cutaneous lesions (90.6 %), oedema or swelling (43.8 %) and destructive changes or remodelling of adjacent bone (42.2 %). Compared with patients in the group >= 6 months, patients in the group < 6 months have higher odds of KMP (P = 0.000), infiltrative lesion with ill-defined borders (P = 0.044). The group >= 6 months have higher odds of destructive changes or remodelling of adjacent bone (P = 0.002). In all patients, the lesions in all of the 64 patients were hypointense or isointense compared with muscle on T1-weighted sequences, and hyperintense on T2-weighted or inversion-recovery sequences, nine patients (14.1 %) showed vascularity. There were 28 patients (43.8 %) with characteristic enhancing and infiltrative soft-tissue thickening. Conclusions Presence of visible cutaneous lesions with ill-defined borders, destructive changes or remodelling of adjacent bone, severe thrombocytopenia and consumptive coagulopathy should favour the diagnosis of KHE.

Automated summary

Kaposiform haemangioendothelioma (KHE) is a rare and locally aggressive disorder with specific clinical and imaging features, including visible cutaneous lesions, destructive changes in adjacent bone, severe thrombocytopenia, and consumptive coagulopathy, which can aid in the diagnosis of this condition.