Musculoskeletal complication in kaposiform hemangioendothelioma without Kasabach–Merritt phenomenon: clinical characteristics and management

Abstract

PurposeMusculoskeletal 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 methodsWe 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.ResultsThe 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.ConclusionMusculoskeletal 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.

Tacrolimus ointment for the treatment of superficial kaposiform hemangioendothelioma and tufted angioma

“…Pathologically, untreated KHE are characterized by progressive fibrosis, and lesions on extremities can lead to contractures . One interesting finding of our study was that impressive improvements were seen in two patients associated with decreased range of motion.…”

“…In addition, superficial lesions located on or adjacent to joints can impair function and lead to decreased range of motion. 2 We show here for the first time that patients with superficial KHE/TA had significant clinical improvements after topical tacrolimus treatment. Like sirolimus, tacrolimus appears to stabilize KHE/TA in their growth phase.…”

“…Although many of these lesions do not lead to life‐threatening complications, some superficial KHE/TA can grow dramatically and destroy tissue, and leave irreversible skin changes. In addition, superficial lesions located on or adjacent to joints can impair function and lead to decreased range of motion …”

“…In addition, the associated musculoskeletal complications (e.g. decreased range of motion) can dramatically reduce patients’ quality of life . Currently, the standard treatment protocols for KHE/TA have yet to be established.…”

“…decreased range of motion) can dramatically reduce patients' quality of life. 2 Currently, the standard treatment protocols for KHE/TA have yet to be established. It is still unknown which agent or combination of agents is optimal.…”