Musculoskeletal complication in kaposiform hemangioendothelioma without Kasabach–Merritt phenomenon: clinical characteristics and management
Authors Ji Y, Yang K, Chen S, Peng S, Lu G, Liu X
Received 15 April 2018
Accepted for publication 25 June 2018
Published 7 September 2018 Volume 2018:10 Pages 3325—3331
Checked for plagiarism Yes
Review by Single anonymous peer review
Peer reviewer comments 2
Editor who approved publication: Dr Kenan Onel
Yi Ji,1 Kaiying Yang,1 Siyuan Chen,2 Suhua Peng,1 Guoyan Lu,3 Xingtao Liu4
1Division of Oncology, Department of Pediatric Surgery, West China Hospital of Sichuan University, Chengdu, China; 2Pediatric Intensive Care Unit, Department of Critical Care Medicine, West China Hospital of Sichuan University, Chengdu, China; 3Department of Pediatrics, Pediatric Intensive Care Unit, West China Second University Hospital, Sichuan University, Chengdu, China; 4Department of Vascular and Interventional Radiology, Chengdu Women and Children’s Central Hospital, Chengdu, China
Purpose: Musculoskeletal complications have been associated with kaposiform hemangioendothelioma (KHE) and can lead to disability and reduced quality of life. We aimed to determine the clinical characteristics of musculoskeletal complication in patients with KHE without Kasabach–Merritt phenomenon (KMP) in order to identify features that may aid clinicians in KHE treatment.
Patients and methods: We conducted a cohort study of KHE without KMP associated with musculoskeletal complication between January 2006 and February 2017 at three tertiary medical centers in China. The study included 29 nonthrombocytopenic patients with KHE and musculoskeletal complication.
Results: The mean age at diagnosis of KHE was 4.5 years (range, 0.3–50.0 years). The mean follow-up was 4.1 years (range, 0.5–9.0 years). In most cases (72.4%), decreased range of motion (ROM) appeared within 2 years of KHE onset. Associated chronic pain was reported in 12 patients. Bone–joint changes were common in patients with decreased ROM (75.9%). All the patients received at least one medical therapy including corticosteroids, vincristine, propranolol, and sirolimus. Sirolimus demonstrated the highest efficacy rate, with 94.7% of patients showing improvements in ROM and chronic pain.
Conclusion: Musculoskeletal complication can occur early in the disease course of KHE without KMP. Although no uniformly effective treatment modality was found, sirolimus demonstrated the best response in patients with KHE with decreased ROM and chronic pain.
Keywords: kaposiform hemangioendothelioma, musculoskeletal disorders, clinical characteristics, complication, sirolimus
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