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Article Dans Une Revue Journal of Neuro-Oncology Année : 2018

Pazopanib efficacy in recurrent central nervous system hemangiopericytomas

Résumé

INTRODUCTION: There is currently no treatment for solitary fibrous tumors/hemangiopericytomas (SFT/H) of the central nervous system recurring after multiple surgeries and radiotherapies. The NAB2-STAT6 gene fusion is the hallmark of these tumors, and upregulates Early Growth Factor, activating several growth pathways. METHODS: We treated two patients presenting pluri-recurrent meningeal SFT/H with Pazopanib, a broad-spectrum tyrosine kinase inhibitor. We analyzed the exome and RNA sequencing data of one of them and, in addition to another meningeal SFT/H, compared it to the transcriptomic profiling of 5 systemic SFT/H. RESULTS: A dramatic clinical and radiological response was observed in both cases, respectively 84 and 43% decrease after 3 months. As a comparison, Pazopanib has only a stabilizing effect in systemic SFT/H. Indeed, central nervous system SFT/H show overexpression of different tyrosine kinases targeted by Pazopanib. CONCLUSIONS: Two consecutive patients with untreatable central nervous system SFT/H showed a spectacular partial response to Pazopanib, an unprecedented result in SFT/H. This result could be explained by differences in expression profiles and calls for a confirmation in a larger cohort of patients.
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Dates et versions

hal-01992423 , version 1 (24-01-2019)

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Caroline Apra, Agusti Alentorn, Karima Mokhtari, Michel Kalamarides, Marc Sanson. Pazopanib efficacy in recurrent central nervous system hemangiopericytomas. Journal of Neuro-Oncology, 2018, 139 (2), pp.369-372. ⟨10.1007/s11060-018-2870-0⟩. ⟨hal-01992423⟩
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