Abstract
ObjectiveTo document the epidemiological, clinical, histological and radiological characteristics of aggressive vascular abnormalities of bone in children.Study designCorrespondents of the French Society of Childhood Malignancies were asked to notify all cases of aggressive vascular abnormalities of bone diagnosed between January 1988 and September 2009.Results21 cases were identified; 62% of the patients were boys. No familial cases were observed, and the disease appeared to be sporadic. Mean age at diagnosis was 8.0 years [0.8-16.9 years]. Median follow-up was 3 years [0.3-17 years]. The main presenting signs were bone fracture (n = 4) and respiratory distress (n = 7), but more indolent onset was observed in 8 cases. Lung involvement, with lymphangiectasies and pleural effusion, was the most frequent form of extraosseous involvement (10/21). Bisphosphonates, alpha interferon and radiotherapy were used as potentially curative treatments. High-dose radiotherapy appeared to be effective on pleural effusion but caused major late sequelae, whereas antiangiogenic drugs like alpha interferon and zoledrenate have had a limited impact on the course of pulmonary complications. The impact of bisphosphonates and alpha interferon on bone lesions was also difficult to assess, owing to insufficient follow-up in most cases, but it was occasionally positive. Six deaths were observed and the overall 10-year mortality rate was about 30%. The prognosis depended mainly on pulmonary and spinal complications.ConclusionAggressive vascular abnormalities of bone are extremely rare in childhood but are lifethreatening. The impact of anti-angiogenic drugs on pulmonary complications seems to be limited, but they may improve bone lesions.
Highlights
Aggressive vascular abnormalities of bone consist of intra-osseous vascular abnormalities leading to osteolysis and, sometimes, extension to adjacent tissues [1]
Patients and Methods All children with a diagnosis of aggressive vascular abnormalities of bone between 1 January 1988 and 1 September 2009 and managed in a French hospital were eligible for the study
The diagnosis of aggressive vascular abnormalities of bone was based on histological examination of a bone lesion
Summary
Aggressive vascular abnormalities of bone consist of intra-osseous vascular abnormalities leading to osteolysis and, sometimes, extension to adjacent tissues [1]. These diseases are very rare, and affect children and young adults [2]. They may be disseminated or focal, but both forms can be very aggressive and life-threatening. The natural history of such diseases is mainly known through individual case reports, and there are only two series with more than patients (15 and cases) [13,14]. We report the epidemiological, clinical and biological characteristics, treatment modalities and outcome
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