A POETIC Phase II study of continuous oral everolimus in recurrent, radiographically progressive pediatric low-grade glioma.


Journal

Pediatric blood & cancer
ISSN: 1545-5017
Titre abrégé: Pediatr Blood Cancer
Pays: United States
ID NLM: 101186624

Informations de publication

Date de publication:
02 2021
Historique:
received: 02 05 2020
revised: 16 10 2020
accepted: 19 10 2020
pubmed: 4 11 2020
medline: 17 6 2021
entrez: 3 11 2020
Statut: ppublish

Résumé

To evaluate efficacy, pharmacokinetics (PK) and pharmacodynamics of single-agent everolimus in pediatric patients with radiographically progressive low-grade glioma (LGG). Everolimus was administered at 5 mg/m Twenty-three eligible patients (median age 9.2 years) were enrolled. All patients received prior chemotherapy (median number of prior regimens two) and/or radiotherapy (two patients). By week 48, two patients had a partial response, 10 stable disease, and 11 clinical or radiographic progression; two discontinued study prior to 1 year (toxicity: 1, physician determination: 1). With a median follow up of 1.8 years (range 0.2-6.7 years), the 2-, 3-, and 5-year progression-free survivals (PFS) were 39 ± 11%, 26 ± 11%, and 26 ± 11%, respectively; two patients died of disease. The 2-, 3-, and 5-year overall survival (OS) were all 93 ± 6%. Grade 1 and 2 toxicities predominated; two definitively related grade 3 toxicities (mucositis and neutropenia) occurred. Grade 4 elevation of liver enzymes was possibly related in one patient. Predose blood levels showed substantial variability between patients with 45.5% below and 18.2% above the target range of 5-15 ng/mL. Pharmacodynamic analysis demonstrated significant inhibition in phospho-S6, 4E-BP1, and modulation of c-Myc expression. Daily oral everolimus provides a well-tolerated, alternative treatment for multiple recurrent, radiographically progressive pediatric LGG. Based on these results, everolimus is being investigated further for this patient population.

Sections du résumé

BACKGROUND
To evaluate efficacy, pharmacokinetics (PK) and pharmacodynamics of single-agent everolimus in pediatric patients with radiographically progressive low-grade glioma (LGG).
METHODS
Everolimus was administered at 5 mg/m
RESULTS
Twenty-three eligible patients (median age 9.2 years) were enrolled. All patients received prior chemotherapy (median number of prior regimens two) and/or radiotherapy (two patients). By week 48, two patients had a partial response, 10 stable disease, and 11 clinical or radiographic progression; two discontinued study prior to 1 year (toxicity: 1, physician determination: 1). With a median follow up of 1.8 years (range 0.2-6.7 years), the 2-, 3-, and 5-year progression-free survivals (PFS) were 39 ± 11%, 26 ± 11%, and 26 ± 11%, respectively; two patients died of disease. The 2-, 3-, and 5-year overall survival (OS) were all 93 ± 6%. Grade 1 and 2 toxicities predominated; two definitively related grade 3 toxicities (mucositis and neutropenia) occurred. Grade 4 elevation of liver enzymes was possibly related in one patient. Predose blood levels showed substantial variability between patients with 45.5% below and 18.2% above the target range of 5-15 ng/mL. Pharmacodynamic analysis demonstrated significant inhibition in phospho-S6, 4E-BP1, and modulation of c-Myc expression.
CONCLUSION
Daily oral everolimus provides a well-tolerated, alternative treatment for multiple recurrent, radiographically progressive pediatric LGG. Based on these results, everolimus is being investigated further for this patient population.

Identifiants

pubmed: 33140540
doi: 10.1002/pbc.28787
pmc: PMC9161236
mid: NIHMS1794283
doi:

Substances chimiques

Antineoplastic Agents 0
Everolimus 9HW64Q8G6G
MTOR protein, human EC 2.7.1.1
TOR Serine-Threonine Kinases EC 2.7.11.1

Types de publication

Clinical Trial, Phase II Journal Article Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

e28787

Subventions

Organisme : NCI NIH HHS
ID : P30 CA008748
Pays : United States
Organisme : NCI NIH HHS
ID : P50 CA165962
Pays : United States

Informations de copyright

© 2020 Wiley Periodicals LLC.

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Auteurs

Karen D Wright (KD)

Dana-Farber/Boston Children's Cancer and Blood Disorders Center, 450 Brookline Avenue, Boston, Massachusetts, 02215, USA.

Xiaopan Yao (X)

Dana-Farber/Boston Children's Cancer and Blood Disorders Center, 450 Brookline Avenue, Boston, Massachusetts, 02215, USA.

Wendy B London (WB)

Dana-Farber/Boston Children's Cancer and Blood Disorders Center, 450 Brookline Avenue, Boston, Massachusetts, 02215, USA.

Pei-Chi Kao (PC)

Dana-Farber/Boston Children's Cancer and Blood Disorders Center, 450 Brookline Avenue, Boston, Massachusetts, 02215, USA.

Lia Gore (L)

Children's Hospital Colorado, 13123 E 16th Avenue, Aurora, Colorado, 80045, USA.

Stephen Hunger (S)

Children's Hospital Colorado, 13123 E 16th Avenue, Aurora, Colorado, 80045, USA.

Russ Geyer (R)

Seattle Children's Hospital, 4800 Sand Point Way NE, Seattle, Washington, 98105, USA.

Kenneth J Cohen (KJ)

The Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins, 401 N Broadway, Baltimore, Maryland, 21231, USA.

Jeffrey C Allen (JC)

New York University Medical Center, 550 1st Avenue, New York, New York, 10016, USA.

Howard M Katzenstein (HM)

Children's Healthcare of Atlanta, 1365 Clifton Road NE, Atlanta, Georgia, 30322, USA.

Amy Smith (A)

University of Florida Health Shands Cancer Hospital, 1515 SW Archer Road, Gainesville, Florida, 32608, USA.

Jessica Boklan (J)

Phoenix Children's Hospital, 1919 E Thomas Rd, Phoenix, Arizona, 85016, USA.

Kellie Nazemi (K)

OHSU Doernbecher Children's Hospital, 700 SW Campus Drive, Portland, Oregon, 97239, USA.

Tanya Trippett (T)

Memorial Sloan Kettering Cancer Center, 1275 York Avenue, New York, New York, 10065, USA.

Matthias Karajannis (M)

Memorial Sloan Kettering Cancer Center, 1275 York Avenue, New York, New York, 10065, USA.

Cynthia Herzog (C)

MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, Texas, 77030, USA.

Joseph Destefano (J)

Memorial Sloan Kettering Cancer Center, 1275 York Avenue, New York, New York, 10065, USA.

Jennifer Direnzo (J)

Memorial Sloan Kettering Cancer Center, 1275 York Avenue, New York, New York, 10065, USA.

Jay Pietrantonio (J)

Dana-Farber/Boston Children's Cancer and Blood Disorders Center, 450 Brookline Avenue, Boston, Massachusetts, 02215, USA.

Lianne Greenspan (L)

Dana-Farber/Boston Children's Cancer and Blood Disorders Center, 450 Brookline Avenue, Boston, Massachusetts, 02215, USA.

Danielle Cassidy (D)

Children's Hospital Colorado, 13123 E 16th Avenue, Aurora, Colorado, 80045, USA.

Debra Schissel (D)

Children's Hospital Colorado, 13123 E 16th Avenue, Aurora, Colorado, 80045, USA.

John Perentesis (J)

Cincinnati Children's Hospital Medical Center, 3333 Burnet Avenue, Cincinnati, Ohio, 45229, USA.

Mitali Basu (M)

Cincinnati Children's Hospital Medical Center, 3333 Burnet Avenue, Cincinnati, Ohio, 45229, USA.

Tomoyuki Mizuno (T)

Cincinnati Children's Hospital Medical Center, 3333 Burnet Avenue, Cincinnati, Ohio, 45229, USA.

Alexander A Vinks (AA)

Cincinnati Children's Hospital Medical Center, 3333 Burnet Avenue, Cincinnati, Ohio, 45229, USA.

Sanjay P Prabhu (SP)

Boston Children's Hospital, 300 Longwood Avenue, Boston, Massachusetts, 02115, USA.

Susan N Chi (SN)

Dana-Farber/Boston Children's Cancer and Blood Disorders Center, 450 Brookline Avenue, Boston, Massachusetts, 02215, USA.

Mark W Kieran (MW)

Dana-Farber/Boston Children's Cancer and Blood Disorders Center, 450 Brookline Avenue, Boston, Massachusetts, 02215, USA.

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