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Article Dans Une Revue Orphanet Journal of Rare Diseases Année : 2010

Retrospective French nationwide survey of childhood aggressive vascular anomalies of bone, 1988-2009.

Sébastien Héritier
  • Fonction : Auteur
Francis Jaubert
  • Fonction : Auteur
Michèle Bigorre
  • Fonction : Auteur
Marion Gillibert-Yvert
  • Fonction : Auteur
Benoit de Courtivron
  • Fonction : Auteur
Makram Ziade
  • Fonction : Auteur
Christian Carrie
  • Fonction : Auteur
Cécile Bost-Bru
  • Fonction : Auteur
Jean-Claude Léonard
  • Fonction : Auteur
Marie Ouache
  • Fonction : Auteur
Liliane Boccon-Gibod
  • Fonction : Auteur
Pierre Mary
  • Fonction : Auteur
Yann Revillon
  • Fonction : Auteur
Véronique Houdoin
  • Fonction : Auteur
Véronique Forin
  • Fonction : Auteur
Hubert Ducou Lepointe
  • Fonction : Auteur
Jane Languepin
  • Fonction : Auteur
Jeanne Wagnon
  • Fonction : Auteur

Résumé

OBJECTIVE: To document the epidemiological, clinical, histological and radiological characteristics of aggressive vascular abnormalities of bone in children. STUDY DESIGN: Correspondents 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. RESULTS: 21 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. CONCLUSION: Aggressive 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.

Dates et versions

hal-00474834 , version 1 (21-04-2010)

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Citer

Sébastien Héritier, Martine Le Merrer, Francis Jaubert, Michèle Bigorre, Marion Gillibert-Yvert, et al.. Retrospective French nationwide survey of childhood aggressive vascular anomalies of bone, 1988-2009.. Orphanet Journal of Rare Diseases, 2010, 5, pp.3. ⟨10.1186/1750-1172-5-3⟩. ⟨hal-00474834⟩
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