Efficacy of vinblastine in central nervous system Langerhans cell histiocytosis: a nationwide retrospective study

• DOI: 10.1186/1750-1172-6-83
• Publication Date: 2011-12-13T07:28:36Z
• Authors: Sophie Ng Wing Tin; Nadine Martin-Duv...; Ahmed Idbaih; Catherine Garel; Maria Ribeiro; Judith Landman Pa...; Anne-Sophie Defac...; Anne Lambilliotte; Mohamed Barkaoui; Martine Munzer; Martine Gardembas; Jean Sibilia; Patrick Lutz; Renato Fior; Michel Polak; Alain Robert; Olivier Aumaitre; Dominique Plantaz; Corinne Armari-Alla; Thierry Genereau; Perrine Marec Berard; Ghislain Nokam Talom; Jean-Loup Pennaforte; Hubert Ducou Le P...; Marie-Anne Barthez; Gérard Couillault; Julien Haroche; Karima Mokhtari; Jean Donadieu; Khê Hoang-Xuan
• Keywords: Langerhans Cell Histiocytosis; Concomitant

Abstract Background Vinblastine (VBL) is the standard treatment for systemic Langerhans cell histiocytosis (LCH), but little known about its efficacy in central nervous system (CNS) mass lesions. Methods A retrospective chart review was conducted. Twenty patients from French LCH Study Group register met inclusion criteria. In brief, they had CNS lesions, been treated with VBL, and were evaluable radiologic response. Results The median age at diagnosis of 11.5 years (range: 1-50). Intravenous VBL 6 mg/m 2 given a 6-week induction treatment, followed by maintenance treatment. total duration 12 months 3-30). Eleven received steroids concomitantly. Fifteen achieved an objective response; five complete response (CR: 25%), ten partial (PR: 50%), four stable disease (SD: 20%) one patient progressed (PD: 5%). Of interest, out six who without concomitant With follow-up 6.8 years, 5-year event-free overall survival 61% 84%, respectively. well-tolerated there no withdrawals due to adverse events. Conclusion or steroids, could potentially be useful therapeutic option especially those inoperable lesions multiple Prospective clinical trials are warranted evaluation this indication.