A phase I/II trial and pharmacokinetic study of mithramycin in children and adults with refractory Ewing sarcoma and EWS-FLI1 fusion transcript.

Purpose: In a preclinical drug screen, mithramycin was identified as a potent inhibitor of the Ewing sarcoma EWS-FLI1 transcription factor. We conducted a phase I/II trial to determine the dose-limiting toxicities (DLT), maximum tolerated dose (MTD), and pharmacokinetics (PK) of mithramycin in child...

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Publicado en:Cancer Chemotherapy & Pharmacology Vol. 80; no. 3; pp. 645 - 653
Autores principales: Grohar, Patrick, Glod, John, Peer, Cody, Sissung, Tristan, Arnaldez, Fernanda, Long, Lauren, Figg, William, Whitcomb, Patricia, Helman, Lee, Widemann, Brigitte, Grohar, Patrick J, Peer, Cody J, Sissung, Tristan M, Arnaldez, Fernanda I, Figg, William D, Helman, Lee J, Widemann, Brigitte C
Formato: clinical trial research tables/charts Journal Article
Publicado: Springer Nature Sep2017
Acceso en línea:Ver este registro en EBSCOhost
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      dt: Sep2017
      vid: 80
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      pub: Springer Nature
      place: New York, New York
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        atl: A phase I/II trial and pharmacokinetic study of mithramycin in children and adults with refractory Ewing sarcoma and EWS-FLI1 fusion transcript.
      aug:
        au:
          Grohar, Patrick
          Glod, John
          Peer, Cody
          Sissung, Tristan
          Arnaldez, Fernanda
          Long, Lauren
          Figg, William
          Whitcomb, Patricia
          Helman, Lee
          Widemann, Brigitte
          Grohar, Patrick J
          Peer, Cody J
          Sissung, Tristan M
          Arnaldez, Fernanda I
          Figg, William D
          Helman, Lee J
          Widemann, Brigitte C
        affil: Pediatric Oncology Branch, Center for Cancer Research , National Cancer Institute , Bethesda USA
      sug:
        subj:
          Aminoglycosides Therapeutic Use
          Osteosarcoma Drug Therapy
          Proteins Metabolism
          Antibiotics, Antineoplastic Therapeutic Use
          Female
          Adolescence
          Human
          Child
          Antibiotics, Antineoplastic Pharmacokinetics
          Male
          Aminoglycosides Pharmacokinetics
          Adult
          Young Adult
          Osteosarcoma Pathology
          Clinical Trials
          Validation Studies
          Comparative Studies
          Evaluation Research
          Multicenter Studies
          Adolescent: 13-18 years
          Child: 6-12 years
          Adult: 19-44 years
          Female
          Male
      ab: Purpose: In a preclinical drug screen, mithramycin was identified as a potent inhibitor of the Ewing sarcoma EWS-FLI1 transcription factor. We conducted a phase I/II trial to determine the dose-limiting toxicities (DLT), maximum tolerated dose (MTD), and pharmacokinetics (PK) of mithramycin in children with refractory solid tumors, and the activity in children and adults with refractory Ewing sarcoma.Patients and Methods: Mithramycin was administered intravenously over 6 h once daily for 7 days for 28 day cycles. Adult patients (phase II) initially received mithramycin at the previously determined recommended dose of 25 µg/kg/dose. The planned starting dose for children (phase I) was 17.5 µg/kg/dose. Plasma samples were obtained for mithramycin PK analysis.Results: The first two adult patients experienced reversible grade 4 alanine aminotransferase (ALT)/aspartate aminotransferase (AST) elevation exceeding the MTD. Subsequent adult patients received mithramycin at 17.5 µg/kg/dose, and children at 13 µg/kg/dose with dexamethasone pretreatment. None of the four subsequent adult and two pediatric patients experienced cycle 1 DLT. No clinical responses were observed. The average maximal mithramycin plasma concentration in four patients was 17.8 ± 4.6 ng/mL. This is substantially below the sustained mithramycin concentrations ≥50 nmol/L required to suppress EWS-FLI1 transcriptional activity in preclinical studies. Due to inability to safely achieve the desired mithramycin exposure, the trial was closed to enrollment.Conclusions: Hepatotoxicity precluded the administration of a mithramycin at a dose required to inhibit EWS-FLI1. Evaluation of mithramycin in patients selected for decreased susceptibility to elevated transaminases may allow for improved drug exposure.
      pubtype: Academic Journal
      doctype:
        clinical trial
        research
        tables/charts
        Journal Article
      ougenre: Article
    language: English
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