Table 3 Clinical trials of systemic treatment for meningioma

Hydroxyurea

retrospective case series

recurrent WHO grade 1 meningioma

60

Duration of stable disease: 3–12 months (median 4.0 months)²⁸⁶

Hydroxyurea

retrospective case series

recurrent WHO grade 2/3 meningioma

35

6-month PFS: 3.0% (median PFS 2.0 months)⁴²⁸

Interferon-α

Phase 2

Recurrent grade 1

35

6-month and 12-month PFS: 54%, 31%; mOS: 8 months⁴²⁹

Interferon-α

Retrospective case series

Recurrent WHO grade 2/3

35

6-month PFS: 17%⁴³⁰

Bevacizumab

retrospective review

recurrent meningioma

14

6-month PFS: 86%⁴³¹

Bevacizumab

retrospectively study

Atypical and anaplastic meningiomas

15

mPFS: 26 weeks. 6-month PFS: 43.8 %²⁹⁰

Mifepristone

Phase III

unresectable meningioma

164

Failure-free and OS were no statistical difference between mifepristone and placebo²⁸⁸

Pasireotide LAR

phase II

recurrent or progressive meningioma

34

It has limited efficacy in recurrent meningiomas²⁴⁴

Octreotide

phase II

recurrent high-grade meningioma

9

6-month PFS: 44.4 %, mPFS: 4.23 months⁴³²

Sandostatin LAR

prospective pilot trial

recurrent meningiomas

16

6-month PFS: 44%, mOS: 7.5 months⁴³³

Temozolomide

Phase II

refractory meningioma

16

Time to tumor progression: 2.5–5.0 months (median 5.0 months); OS: 4–9 months (median 7.5 months)⁴³⁴

Trabectedin

phase II

recurrent WHO grade 2 or 3 meningioma

90

not improve PFS and OS⁴³⁵

Octreotide and everolimus

phase II CEVOREM trial

recurrent meningiomas

20

6-month PFS: 55%, and OS 6- and 12-month were 90 and 75%, respectively³⁰⁸

Everolimus and bevacizumab

phase II

recurrent meningioma

18

median duration of disease stabilization: 10 months⁴³⁶

Sunitinib

phase II

recurrent WHO grades 2–3 meningioma

36

mPFS: 5.2 months, and mOS: 24.6 months³⁰⁷

Nivolumab

phase II

recurrent atypical/anaplastic meningioma

25

6-month PFS: 42.4%; mOS: 30.9 months; One patient achieved radiographic response (ongoing at 4.5 years).²⁹³