%0 Journal Article
%A Steinbügl, Mona
%A Nemes, Karolina
%A Johann, Pascal
%A Kröncke, Thomas
%A Tüchert, Stefanie
%A da Costa, Maria Joao Gil
%A Ebinger, Martin
%A Schüller, Ulrich
%A Sehested, Astrid
%A Hauser, Peter
%A Reinhard, Harald
%A Sumerauer, David
%A Hettmer, Simone
%A Jakob, Marcus
%A Hasselblatt, Martin
%A Siebert, Reiner
%A Witt, Olaf
%A Gerss, Joachim
%A Kerl, Kornelius
%A Frühwald, Michael C
%T Clinical evidence for a biological effect of epigenetically active decitabine in relapsed or progressive rhabdoid tumors.
%J Pediatric blood & cancer
%V 68
%N 12
%@ 1545-5017
%C New York, NY
%I Wiley
%M DKFZ-2021-01755
%P e29267
%D 2021
%Z 2021 Dec;68(12):e29267
%X Refined therapy has helped to improve survival rates in rhabdoid tumors (RT). Prognosis for patients with chemoresistant, recurrent, or progressive RT remains dismal. Although decitabine, an epigenetically active agent, has mainly been evaluated in the management of hematologic malignancies in adults, safety in children has also been demonstrated repeatedly.A retrospective series of patients who received decitabine upon relapse or progression following therapy according to the EU-RHAB regimen is presented. Due to the retrospective nature of analyses, response was defined as measurable regression of at least one lesion on imaging. 850k methylation profiling was done whenever tumor tissue was available.A total of 22 patients with RT of any anatomical localization were included. Most patients (19/22) presented with metastases. All received low-dose decitabine with or preceding conventional chemotherapy. Patients received a median of two (1-6) courses of decitabine; 27.3
%K ATRT (Other)
%K decitabine (Other)
%K malignant rhabdoid tumor (Other)
%K relapsed and refractory rhabdoid tumors (Other)
%F PUB:(DE-HGF)16
%9 Journal Article
%$ pmid:34347371
%R 10.1002/pbc.29267
%U https://inrepo02.dkfz.de/record/170092