International Journal of Hematology

DOI: 10.1007/s12185-018-2553-9 Pages: 1-5

A case of recurrent histiocytic sarcoma with MAP2K1 pathogenic variant treated with the MEK inhibitor trametinib

1. National Cancer Center Hospital, Department of Pediatric Oncology

2. National Cancer Center Hospital, Department of Pharmacy

3. National Cancer Hospital, Department of Pathology

4. Nippon Medical School, Department of Pediatrics

Correspondence to:
Tadashi Kumamoto
Tel: +81-3-3542-2511



Histiocytic sarcoma in advanced clinical stages is typically an aggressive neoplasm, with poor response to conventional chemotherapy. An 18-year-old male with refractory histiocytic sarcoma that had transformed from Rosai–Dorfman disease was admitted to our hospital. A pathogenic variant of MAP2K1 was detected by next-generation sequencing of tumor specimens. Affected regions showed excellent responses to the MEK inhibitor trametinib. It has been reported that RAS/MEK/ERK pathway is activated in many cases of histiocytic sarcoma. MEK inhibition may represent a useful treatment option in histiocytic sarcoma.

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