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@ARTICLE{Ugurel:304468,
      author       = {S. Ugurel and N. Abu Rached and T. Gambichler and M.
                      Mengoni and T. Tüting and J. C. Hassel and R. Reschke and
                      G. C. Lodde$^*$ and J.-M. Placke$^*$ and D. Schadendorf$^*$
                      and L. Reinhardt and A. Kreuter and M. Gschnell and J.
                      Utikal$^*$ and M. Erdmann and P. Dücker and D. Helbig and
                      C. Franklin and C. Gebhardt and G. Poch and K. C. Kähler
                      and M. Weichenthal and R. Gutzmer and L. Heinzerling and C.
                      Weishaupt and P. Mohr and K.-M. Thoms and B. Lang and B.
                      Schilling and S. Haferkamp and M. Sachse and J. Welzel and
                      F. Jochims and U. Raap and G. Schley and P. Terheyden and E.
                      Dippel and C. Pöttgen and J. C. Becker$^*$ and A.
                      Tasdogan$^*$ and T. Amaral and L. Nanz and U. Leiter},
      title        = {{O}utcome of systemic therapy in patients with advanced
                      rare skin cancers: {A} retrospective multicenter {D}e{COG}
                      study of 209 patients.},
      journal      = {European journal of cancer},
      volume       = {228},
      issn         = {0959-8049},
      address      = {Amsterdam [u.a.]},
      publisher    = {Elsevier},
      reportid     = {DKFZ-2025-01861},
      pages        = {115750},
      year         = {2025},
      abstract     = {For rare skin cancers, few data exist on the outcome of
                      systemic therapies, particularly immune checkpoint
                      inhibition (ICI). The present study analysed the real-world
                      use of different systemic therapies including ICI, and its
                      outcome in patients with advanced rare skin cancers.This
                      retrospective multicenter study included patients who
                      received systemic therapy for advanced, non-resectable
                      cutaneous angiosarcoma (AS), Kaposi sarcoma (KS),
                      pleomorphic dermal sarcoma (PDS), or cutaneous adnexal
                      carcinoma (CAC). Study endpoints were best overall response
                      (BOR), progression-free survival (PFS) and overall survival
                      (OS).209 patients (77 AS; 81 KS; 14 PDS; and 37 CAC) from 30
                      centers were included. As first-line treatment AS and KS
                      patients predominantly received chemotherapy (77.9 $\%;$
                      63.0 $\%),$ while PDS and CAC patients mostly received ICI
                      (64.4 $\%;$ 43.2 $\%).$ BOR in first-line across all therapy
                      types was 65.5 $\%$ in KS, 50.0 $\%$ in PDS, 41.6 $\%$ in
                      AS, and 10.8 $\%$ in CAC. BOR for ICI was 66.6 $\%$ for PDS,
                      58.3 $\%$ for AS, 33.3 $\%$ for KS, and 4.3 $\%$ for CAC,
                      irrespective of treatment line. 1-year PFS rate upon any
                      first-line therapy was 70.7 $\%$ for PDS, 45.7 $\%$ for KS,
                      25.6 $\%$ for AS, and 18.5 $\%$ for CAC (p < 0.001). 1-year
                      tumor-specific OS rate was 97.3 $\%$ in KS, 84.2 $\%$ in AS,
                      67.7 $\%$ in PDS, and 65.4 $\%$ in CAC (p < 0.001).Type and
                      outcome of systemic therapy differed between cancer
                      entities. Efficacy of ICI was high in PDS and AS, moderate
                      in KS, and low in CAC. Patients with advanced CAC revealed
                      an extremely poor prognosis regardless of the type of
                      therapy used.},
      keywords     = {Angiosarcoma (Other) / Chemotherapy (Other) / Cutaneous
                      adnexal carcinoma (Other) / Immune checkpoint inhibition
                      (Other) / Kaposi sarcoma (Other) / Pleomorphic dermal
                      sarcoma (Other) / Targeted therapy (Other)},
      cin          = {ED01 / A370},
      ddc          = {610},
      cid          = {I:(DE-He78)ED01-20160331 / I:(DE-He78)A370-20160331},
      pnm          = {311 - Zellbiologie und Tumorbiologie (POF4-311)},
      pid          = {G:(DE-HGF)POF4-311},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:40912056},
      doi          = {10.1016/j.ejca.2025.115750},
      url          = {https://inrepo02.dkfz.de/record/304468},
}