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@ARTICLE{Remde:276386,
      author       = {H. Remde and L. Schmidt-Pennington and M. Reuter and L.-S.
                      Landwehr and M. Jensen and H. Lahner and O. Kimpel and B.
                      Altieri and K. Laubner and J. Schreiner and J. Bojunga and
                      S. Kircher and C. A. Kunze and A. Pohrt and M.-V.
                      Teleanu$^*$ and D. Hübschmann$^*$ and A. Stenzinger and H.
                      Glimm$^*$ and S. Fröhling$^*$ and M. Fassnacht and K. Mai
                      and M. Kroiss},
      title        = {{O}utcome of {I}mmunotherapy in {A}drenocortical
                      {C}arcinoma - {A} retrospective cohort study.},
      journal      = {European journal of endocrinology},
      volume       = {188},
      number       = {6},
      issn         = {0001-5598},
      address      = {Bristol},
      publisher    = {BioScientifica Ltd.},
      reportid     = {DKFZ-2023-01083},
      pages        = {485-493},
      year         = {2023},
      note         = {2023 Jun 7;188(6):485-493},
      abstract     = {Clinical trials with immune checkpoint inhibitors (ICI) in
                      adrenocortical carcinoma (ACC) have yielded contradictory
                      results. We aimed to evaluate treatment response and safety
                      of ICI in ACC in a real-life setting.Retrospective cohort
                      study of 54 patients with advanced ACC receiving ICI as
                      compassionate use at six German reference centres between
                      2016 and 2022.Objective response rate (ORR), disease control
                      rate (DCR), progression-free survival (PFS), overall
                      survival (OS) and treatment-related adverse events (TRAE)
                      were assessed.In 52 patients surviving at least 4 weeks
                      after initiation of ICI, ORR was $13.5\%$ (6-26) and DCR
                      $24\%$ (16-41). PFS was 3.0 months $(95\%CI$ 2.3-3.7). In
                      all patients, median OS was 10.4 months (3.8-17). 17 TRAE
                      occurred in 15 patients, which was associated with a longer
                      PFS of 5.5 (1.9-9.2) vs. 2.5 (2.0- 3.0) months (HR 0.29,
                      $95\%CI$ 0.13-0.66, p=0.001) and OS of 28.2 (9.5-46.8) vs.
                      7.0 (4.1-10.2) months (HR 0.34, $95\%CI$ 0.12-0.93).
                      Positive tissue staining for programmed cell death ligand 1
                      (PD-L1) was associated with a longer PFS of 3.2 (2.6-3.8)
                      vs. 2.3 (1.6-3.0, p<0.05) months. Adjusted for concomitant
                      mitotane use, treatment with nivolumab was associated with
                      lower risk of progression (HR 0.36, 0.15-0.90) and death (HR
                      0.20, 0.06-0.72) compared to pembrolizumab.In the real-life
                      setting we observe a response comparable to other
                      second-line therapies and an acceptable safety profile in
                      ACC patients receiving different ICI. The relevance of PD-L1
                      as a marker of response and the potentially more favourable
                      outcome in nivolumab treated patients require confirmation.},
      keywords     = {PD-L1 (Other) / adverse drug reaction (Other) / immune
                      checkpoint inhibitor (Other) / mitotane (Other) / treatment
                      (Other)},
      cin          = {B340 / HD01 / B280 / DD01},
      ddc          = {610},
      cid          = {I:(DE-He78)B340-20160331 / I:(DE-He78)HD01-20160331 /
                      I:(DE-He78)B280-20160331 / I:(DE-He78)DD01-20160331},
      pnm          = {312 - Funktionelle und strukturelle Genomforschung
                      (POF4-312)},
      pid          = {G:(DE-HGF)POF4-312},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:37260092},
      doi          = {10.1093/ejendo/lvad054},
      url          = {https://inrepo02.dkfz.de/record/276386},
}