Efficacy and Safety of Everolimus (RAD001) in Patients of All Ages With Subependymal Giant Cell Astrocytoma Associated With Tuberous Sclerosis Complex (TSC)(EXIST-1)

NCT ID: NCT00789828

Last Updated: 2016-02-03

Study Results

Results available

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Basic Information

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Recruitment Status

COMPLETED

Clinical Phase

PHASE3

Total Enrollment

117 participants

Study Classification

INTERVENTIONAL

Study Start Date

2009-08-31

Study Completion Date

2014-10-31

Brief Summary

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This study evaluated the efficacy and safety of Everolimus in treating patients with Subependymal Giant Cell Astrocytomas associated with Tuberous Sclerosis Complex.

Detailed Description

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Conditions

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Tuberous Sclerosis Subependymal Giant Cell Astrocytoma

Study Design

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Allocation Method

RANDOMIZED

Intervention Model

PARALLEL

Primary Study Purpose

TREATMENT

Blinding Strategy

QUADRUPLE

Participants Caregivers Investigators Outcome Assessors

Study Groups

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Everolimus

Everolimus was administered orally at a starting dose of 4.5mg/m\^2 daily and subsequently titrated to attain whole blood trough concentration of 5 to 15 ng/mL. Dose adjustments were permitted based on safety and whole blood trough concentrations.

Group Type EXPERIMENTAL

Everolimus

Intervention Type DRUG

Everolimus was formulated as tablets of 1.0-mg strength and was blisterpacked under aluminum foil in units of 10 tablets.

Placebo

Matching Placebo administered orally.

Group Type PLACEBO_COMPARATOR

Placebo

Intervention Type DRUG

Placebo was provided as a matching tablet and was blisterpacked under aluminum foil in units of 10.

Interventions

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Everolimus

Everolimus was formulated as tablets of 1.0-mg strength and was blisterpacked under aluminum foil in units of 10 tablets.

Intervention Type DRUG

Placebo

Placebo was provided as a matching tablet and was blisterpacked under aluminum foil in units of 10.

Intervention Type DRUG

Other Intervention Names

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RAD001

Eligibility Criteria

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Inclusion Criteria

* All Ages
* Definite diagnosis of Tuberous Sclerosis according to the modified Gomez criteria
* At least one Subependymal Giant Cell Astrocytoma of at least 1 cm in diameter
* Evidence of SEGA worsening as compared to prior MRI scans
* Females of child bearing potential must use birth control
* Written informed consent

Exclusion Criteria

* SEGA related surgery is likely to be required in the opinion of the investigator
* Recent heart attack, cardiac related chest pain or stroke
* Severely impaired lung function
* Severe liver dysfunction
* Severe kidney dysfunction
* Pregnancy or breast feeding
* Current infection
* History of organ transplant
* Surgery within two months prior to study enrollment
* Prior therapy with a medication in the same class as Everolimus
* Uncontrolled high cholesterol
* Uncontrolled diabetes
* HIV
* Patients with metal implants thus prohibiting MRI evaluations
Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Novartis Pharmaceuticals

INDUSTRY

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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Novartis Pharmaceuticlas

Role: STUDY_DIRECTOR

Novartis Pharmaceuticals

Locations

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University of Alabama at Birmingham

Birmingham, Alabama, United States

Site Status

Barrow Tuberous Sclerosis Center

Phoenix, Arizona, United States

Site Status

University of California at Los Angeles

Los Angeles, California, United States

Site Status

Children's Hospital Oakland Hematology/Oncology Dept

Oakland, California, United States

Site Status

Children's Healthcare of Atlanta

Atlanta, Georgia, United States

Site Status

University of CHicago Comer Children's Hospital

Chicago, Illinois, United States

Site Status

Massachusetts General Hospital Mass General

Boston, Massachusetts, United States

Site Status

Children's Hospital Boston SC-1

Boston, Massachusetts, United States

Site Status

Minnesota Epilepsy Group - PA

Saint Paul, Minnesota, United States

Site Status

Cincinnati Children's Hospital Medical Center Cincinnati Children's Hosp

Cincinnati, Ohio, United States

Site Status

Texas Scottish Rite Hospital for Children

Dallas, Texas, United States

Site Status

Children's Regional Outpatients Center SC

Fairfax, Virginia, United States

Site Status

Novartis Investigative Site

Randwick, New South Wales, Australia

Site Status

Novartis Investigative Site

Brussels, , Belgium

Site Status

Novartis Investigative Site

Toronto, Ontario, Canada

Site Status

Novartis Investigative Site

Québec, , Canada

Site Status

Novartis Investigative Site

Berlin, , Germany

Site Status

Novartis Investigative Site

Heidelberg, , Germany

Site Status

Novartis Investigative Site

Genova, GE, Italy

Site Status

Novartis Investigative Site

Roma, , Italy

Site Status

Novartis Investigative Site

Utrecht, Netherlands, Netherlands

Site Status

Novartis Investigative Site

Warsaw, , Poland

Site Status

Novartis Investigative Site

Moscow, , Russia

Site Status

Novartis Investigative Site

Bristol, , United Kingdom

Site Status

Countries

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United States Australia Belgium Canada Germany Italy Netherlands Poland Russia United Kingdom

References

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Bissler JJ, Budde K, Sauter M, Franz DN, Zonnenberg BA, Frost MD, Belousova E, Berkowitz N, Ridolfi A, Christopher Kingswood J. Effect of everolimus on renal function in patients with tuberous sclerosis complex: evidence from EXIST-1 and EXIST-2. Nephrol Dial Transplant. 2019 Jun 1;34(6):1000-1008. doi: 10.1093/ndt/gfy132.

Reference Type DERIVED
PMID: 30053159 (View on PubMed)

Sparagana S, Franz DN, Krueger DA, Bissler JJ, Berkowitz N, Burock K, Kingswood JC. Pooled analysis of menstrual irregularities from three major clinical studies evaluating everolimus for the treatment of tuberous sclerosis complex. PLoS One. 2017 Oct 12;12(10):e0186235. doi: 10.1371/journal.pone.0186235. eCollection 2017.

Reference Type DERIVED
PMID: 29023494 (View on PubMed)

Franz DN, Belousova E, Sparagana S, Bebin EM, Frost MD, Kuperman R, Witt O, Kohrman MH, Flamini JR, Wu JY, Curatolo P, de Vries PJ, Berkowitz N, Niolat J, Jozwiak S. Long-Term Use of Everolimus in Patients with Tuberous Sclerosis Complex: Final Results from the EXIST-1 Study. PLoS One. 2016 Jun 28;11(6):e0158476. doi: 10.1371/journal.pone.0158476. eCollection 2016.

Reference Type DERIVED
PMID: 27351628 (View on PubMed)

Jozwiak S, Kotulska K, Berkowitz N, Brechenmacher T, Franz DN. Safety of Everolimus in Patients Younger than 3 Years of Age: Results from EXIST-1, a Randomized, Controlled Clinical Trial. J Pediatr. 2016 May;172:151-155.e1. doi: 10.1016/j.jpeds.2016.01.027. Epub 2016 Feb 6.

Reference Type DERIVED
PMID: 26858193 (View on PubMed)

Goyer I, Dahdah N, Major P. Use of mTOR inhibitor everolimus in three neonates for treatment of tumors associated with tuberous sclerosis complex. Pediatr Neurol. 2015 Apr;52(4):450-3. doi: 10.1016/j.pediatrneurol.2015.01.004. Epub 2015 Jan 14.

Reference Type DERIVED
PMID: 25682485 (View on PubMed)

Franz DN, Belousova E, Sparagana S, Bebin EM, Frost M, Kuperman R, Witt O, Kohrman MH, Flamini JR, Wu JY, Curatolo P, de Vries PJ, Berkowitz N, Anak O, Niolat J, Jozwiak S. Everolimus for subependymal giant cell astrocytoma in patients with tuberous sclerosis complex: 2-year open-label extension of the randomised EXIST-1 study. Lancet Oncol. 2014 Dec;15(13):1513-1520. doi: 10.1016/S1470-2045(14)70489-9. Epub 2014 Nov 10.

Reference Type DERIVED
PMID: 25456370 (View on PubMed)

Kingswood JC, Jozwiak S, Belousova ED, Frost MD, Kuperman RA, Bebin EM, Korf BR, Flamini JR, Kohrman MH, Sparagana SP, Wu JY, Brechenmacher T, Stein K, Berkowitz N, Bissler JJ, Franz DN. The effect of everolimus on renal angiomyolipoma in patients with tuberous sclerosis complex being treated for subependymal giant cell astrocytoma: subgroup results from the randomized, placebo-controlled, Phase 3 trial EXIST-1. Nephrol Dial Transplant. 2014 Jun;29(6):1203-10. doi: 10.1093/ndt/gfu013. Epub 2014 Apr 11.

Reference Type DERIVED
PMID: 24729041 (View on PubMed)

Kotulska K, Borkowska J, Jozwiak S. Possible prevention of tuberous sclerosis complex lesions. Pediatrics. 2013 Jul;132(1):e239-42. doi: 10.1542/peds.2012-3607. Epub 2013 Jun 3.

Reference Type DERIVED
PMID: 23733802 (View on PubMed)

Franz DN, Belousova E, Sparagana S, Bebin EM, Frost M, Kuperman R, Witt O, Kohrman MH, Flamini JR, Wu JY, Curatolo P, de Vries PJ, Whittemore VH, Thiele EA, Ford JP, Shah G, Cauwel H, Lebwohl D, Sahmoud T, Jozwiak S. Efficacy and safety of everolimus for subependymal giant cell astrocytomas associated with tuberous sclerosis complex (EXIST-1): a multicentre, randomised, placebo-controlled phase 3 trial. Lancet. 2013 Jan 12;381(9861):125-32. doi: 10.1016/S0140-6736(12)61134-9. Epub 2012 Nov 14.

Reference Type DERIVED
PMID: 23158522 (View on PubMed)

Related Links

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http://www.novartisclinicaltrials.com/webapp/portals/EXISTClinicalTrials/page.do

Visit EXIST-1 NovartisClinicalTrials.com: Pre-qualify for a trial, and view a list of trials and participating study centers.

Other Identifiers

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2007-006997-27

Identifier Type: EUDRACT_NUMBER

Identifier Source: secondary_id

CRAD001M2301

Identifier Type: -

Identifier Source: org_study_id

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