Kaposiform hemangioendothelioma without cutaneous involvement

Of 30 patients (mean age at diagnosis, 55.6 months), 17 (56.7%) were male. Fourteen (46.7%) patients were associated with Kasabach–Merritt phenomenon (KMP). Patients with KMP were significantly more likely to have lesions involving truck compared to patients without KMP (odds ratio 10.000; 95% confidence interval 1.641–60.921; P = 0.011). Other common complication included severe anemia and decreased range of motion. In the majority of cases (93.3%), the lesions were highly infiltrative and locally invasive with ill-defined margins. Histological examination was required in all patients without KMP for precise diagnosis. In all, 16 (53.3%) patients received corticosteroid treatment, 19 (63.3%) received oral sirolimus treatment, 7 (23.3%) received intravenous vincristine, and 5 (16.7%) patients used propranolol. Patients had varied responses to conventional drugs, whereas all patients receiving sirolimus treatment had better response. In all, three patients (10%) died of disease, all presented with KMP. Feature of these recalcitrant cases (death) included young age, visceral location, extensive involvement, and lack of improvement with high-dose corticosteroids.