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. 2011 Dec 12;6:83.
doi: 10.1186/1750-1172-6-83.

Efficacy of Vinblastine in Central Nervous System Langerhans Cell Histiocytosis: A Nationwide Retrospective Study

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Free PMC article

Efficacy of Vinblastine in Central Nervous System Langerhans Cell Histiocytosis: A Nationwide Retrospective Study

Sophie Ng Wing Tin et al. Orphanet J Rare Dis. .
Free PMC article

Abstract

Background: Vinblastine (VBL) is the standard treatment for systemic Langerhans cell histiocytosis (LCH), but little is known about its efficacy in central nervous system (CNS) mass lesions.

Methods: A retrospective chart review was conducted. Twenty patients from the French LCH Study Group register met the inclusion criteria. In brief, they had CNS mass lesions, had been treated with VBL, and were evaluable for radiologic response.

Results: The median age at diagnosis of LCH was 11.5 years (range: 1-50). Intravenous VBL 6 mg/m2 was given in a 6-week induction treatment, followed by a maintenance treatment. The median total duration was 12 months (range: 3-30). Eleven patients received steroids concomitantly. Fifteen patients achieved an objective response; five had a complete response (CR: 25%), ten had a partial response (PR: 50%), four had stable disease (SD: 20%) and one patient progressed (PD: 5%). Of interest, four out of the six patients who received VBL without concomitant steroids achieved an objective response. With a median follow-up of 6.8 years, the 5-year event-free and overall survival was 61% and 84%, respectively. VBL was well-tolerated and there were no patient withdrawals due to adverse events.

Conclusion: VBL, with or without steroids, could potentially be a useful therapeutic option in LCH with CNS mass lesions, especially for those with inoperable lesions or multiple lesions. Prospective clinical trials are warranted for the evaluation of VBL in this indication.

Figures

Figure 1
Figure 1
Complete response to vinblastine chemotherapy. Dura mater lesion. Axial T1-weighted magnetic resonance imaging (MRI) with gadolinium before treatment with vinblastine (A) and 8 months after treatment initiation showing a complete response (B).
Figure 2
Figure 2
Partial response to vinblastine chemotherapy. Hypothalamic and temporal lesions. Coronal T1-weighted magnetic resonance imaging (MRI) with gadolinium before treatment with vinblastine (A) and 12 months after treatment initiation, showing a partial response (B).
Figure 3
Figure 3
Complete response to vinblastine chemotherapy. Third ventricle lesion. Axial T1-weighted magnetic resonance imaging (MRI) with gadolinium before vinblastine treatment (A) and 6 months after treatment initiation, showing a complete response (B).
Figure 4
Figure 4
Event-free survival calculated by the Kaplan-Meier method. Events are progressive disease or any new progression after initial control of the CNS lesion. Duration is expressed in years.

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