Lymphatic Markers in Kaposiform Hemangioendothelioma: Implications for Tumor Invasiveness and β-blockers efficacy

Abstract

Background: Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor that usually expresses lymphatic markers. PROX1 can increase the local invasiveness of KHE in mice. Objectives: To evaluate whether the expression of lymphatic markers in KHE patients increases their invasiveness and decreases the efficacy of β-blockers (propranolol or atenolol). Methods: The tumor tissues of 3 patients with KHE were collected for transcriptomics. The clinical data of 15 patients were collected to analyze the correlation between PROX1, invasiveness and β-blockers’ efficacy. Overexpression PROX1 in EOMAs, cell viability assay, apoptosis, cell cycle, migration, and β-blockers’ efficacy were analyzed in vitro. Tumor invasiveness was analyzed in vivo. Results: PROX1 might increase local invasiveness of the KHE due to impact cell motility, locomotion, and migration. The nearly complete resolution rate (>75%) of β-blockers (propranolol or atenolol) in the treatment of KHE was 40 %. There was no significant correlation between the positive rate of PROX1 and the efficacy of β-blockers’ treatment. Limitations: Due to small numbers, the effect of PROX1 on the efficacy of propranolol needs further study. Conclusion: Our study showed the higher positive rate of PROX1 in the tumor, the more prone the local invasiveness of the KHE patients. There was no significant correlation between the positive rate of PROX1 and the efficacy of 10 patients with β-blockers treatment.