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@ARTICLE{Riedmeier:307581,
      author       = {M. Riedmeier and H. Frey and S. R. Antonini and G. F. L.
                      Canali and C. F. Classen and N. Domínguez-Pinilla and M.
                      Fassnacht and J. Finger and S. Fuchs$^*$ and M. Grönroos
                      and M. P. Halah and C. Härtel and D. Janus and A.
                      Jaspers-Bakker and R. R. de Krijger and T. Kutluk and M.
                      Mezoued and J. Munarin and M. van Noesel and N. Ö. Köse
                      and S. H. Pearce and T. Perwein and S. Puglisi and P.-G.
                      Schlegel and V. Binder-Blaser and G. Tuli and J. Walenciak
                      and B. Yalcin and V. Wiegering},
      title        = {{M}itotane dosage, plasma levels, and anthropometric
                      measurements in pediatric adrenocortical carcinoma.},
      journal      = {Endocrine oncology},
      volume       = {6},
      number       = {1},
      issn         = {2634-4793},
      address      = {[Bristol]},
      publisher    = {Bioscientifica Ltd.},
      reportid     = {DKFZ-2026-00076},
      pages        = {e240081},
      year         = {2026},
      note         = {#DKTKZFB9#},
      abstract     = {Mitotane is an effective treatment for advanced
                      adrenocortical carcinoma (ACC). Given the limited pediatric
                      data available, this study aims to evaluate the associations
                      between mitotane dosage, plasma drug levels, and
                      anthropometric measurements, as well as their potential
                      impact on dosage requirements to optimize therapeutic
                      outcomes in pediatric patients with ACC (pACC).A
                      retrospective, international, multicenter study was
                      conducted on pediatric ACC patients treated with mitotane
                      across 18 centers. Mitotane serum levels were obtained from
                      the Lysosafe Online® database or directly from the centers.
                      Data from the cohort with plasma levels within the target
                      range (≥14 mg/L; n = 319) were analyzed and compared to
                      those with levels outside this range (n = 320).Fifty
                      pediatric patients $(60\%$ female) diagnosed between 2004
                      and 2023 were included, with a median follow-up of 34.5
                      months and a 10-year overall survival of 33 months. The
                      median age at diagnosis was 8.6 years, with most tumors
                      $(84\%)$ hormone-secreting. Among 49 patients undergoing
                      surgery, 31 $(62\%)$ achieved R0 resection. The median
                      treatment duration was 18 months, with a median mitotane
                      dose of 87 mg/kg/day in patients within the target plasma
                      level range, showing no significant difference from those
                      outside the range. However, BMI was significantly associated
                      with doses of plasma levels in target range (P = 0.001), as
                      underweight (105.4 mg/kg/day) and healthy weight patients
                      (98.4 mg/kg/day) required higher doses than overweight/obese
                      patients (44.4 mg/kg/day). No significant differences in
                      daily dose levels (mg/kg/day and mg/m2/day) were observed
                      based on body weight.This study supports estimating mitotane
                      dosages in pediatric ACC, emphasizing the need for close
                      monitoring and frequent follow-ups at specialized centers
                      due to individualized dosing and a narrow therapeutic
                      window.},
      keywords     = {mitotane (Other) / mitotane dosage (Other) / mitotane
                      plasma level (Other) / pediatric adrenocortical carcinoma
                      (Other) / pediatric adrenocortical tumor (Other)},
      cin          = {BE01},
      ddc          = {610},
      cid          = {I:(DE-He78)BE01-20160331},
      pnm          = {899 - ohne Topic (POF4-899)},
      pid          = {G:(DE-HGF)POF4-899},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:41503252},
      pmc          = {pmc:PMC12771559},
      doi          = {10.1530/EO-24-0081},
      url          = {https://inrepo02.dkfz.de/record/307581},
}