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Peer-reviewedCC BYAnnals of hematology

Post-transplant BRAF/MEK inhibitor maintenance sustains complete remission in BRAF V600E-mutant histiocytic sarcoma.

4 affiliations
  1. 1Department of Hematology and Oncology, Carreras Leukämie Centrum, Philipps-Universität Marburg, University Hospital Giessen and Marburg, Marburg, Germany. [email protected].
  2. 2Department of Hematology and Oncology, University Hospital Giessen and Marburg, Giessen, Germany.
  3. 3Institute of Pathology, University Hospital Giessen and Marburg, Giessen, Germany.
  4. 4Department of Hematology and Oncology, Carreras Leukämie Centrum, Philipps-Universität Marburg, University Hospital Giessen and Marburg, Marburg, Germany.

Abstract

Histiocytic sarcoma is a rare and aggressive malignancy with poor outcomes under conventional chemotherapy. Activating MAPK pathway alterations, including BRAF V600E mutations, provide a rationale for targeted therapy; however, the durability of responses and the role of post-transplant maintenance remain unclear. We report a patient with refractory BRAF V600E-mutant histiocytic sarcoma who achieved complete remission following BRAF/MEK inhibition and subsequently underwent allogeneic stem cell transplantation. Post-transplant maintenance therapy with dabrafenib and trametinib was initiated early after engraftment. The patient achieved sustained complete remission with no radiological or molecular evidence of disease during follow-up exceeding one year. Circulating tumor DNA analysis remained negative for previously detected mutations. This case suggests that an integrated strategy combining targeted therapy, allogeneic transplantation, and post-transplant maintenance may enable durable disease control in high-risk BRAF-mutant histiocytic sarcoma and warrants further evaluation.

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BibTeX
@article{karim2026posttransplant,
  title = {{Post-transplant BRAF/MEK inhibitor maintenance sustains complete remission in BRAF V600E-mutant histiocytic sarcoma.}},
  author = {Ihab Karim and Tobias Arnold and Andreas Bräuninger and Marius Dohse and Felix Schell and Maisun Abu Samra and Thomas Tarawneh and Andreas Burchert and Stefan Gattenlöhner and Kristina Sohlbach},
  journal = {Annals of hematology},
  year = {2026},
  month = oct,
  doi = {10.1007/s00277-026-07277-6},
  url = {https://europepmc.org/article/MED/42957845},
  note = {CC BY},
}
RIS
TY  - JOUR
TI  - Post-transplant BRAF/MEK inhibitor maintenance sustains complete remission in BRAF V600E-mutant histiocytic sarcoma.
AU  - Ihab Karim
AU  - Tobias Arnold
AU  - Andreas Bräuninger
AU  - Marius Dohse
AU  - Felix Schell
AU  - Maisun Abu Samra
AU  - Thomas Tarawneh
AU  - Andreas Burchert
AU  - Stefan Gattenlöhner
AU  - Kristina Sohlbach
PY  - 2026
DA  - 2026/10/28
JO  - Annals of hematology
DO  - 10.1007/s00277-026-07277-6
UR  - https://europepmc.org/article/MED/42957845
AB  - Histiocytic sarcoma is a rare and aggressive malignancy with poor outcomes under conventional chemotherapy. Activating MAPK pathway alterations, including BRAF V600E mutations, provide a rationale for targeted therapy; however, the durability of responses and the role of post-transplant maintenance remain unclear. We report a patient with refractory BRAF V600E-mutant histiocytic sarcoma who achieved complete remission following BRAF/MEK inhibition and subsequently underwent allogeneic stem cell transplantation. Post-transplant maintenance therapy with dabrafenib and trametinib was initiated early after engraftment. The patient achieved sustained complete remission with no radiological or molecular evidence of disease during follow-up exceeding one year. Circulating tumor DNA analysis remained negative for previously detected mutations. This case suggests that an integrated strategy combining targeted therapy, allogeneic transplantation, and post-transplant maintenance may enable durable disease control in high-risk BRAF-mutant histiocytic sarcoma and warrants further evaluation.
SN  - 0939-5555
SN  - 1432-0584
ER  -