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International Journal of Hematology

, Volume 109, Issue 2, pp 228–232 | Cite as

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

  • Tadashi KumamotoEmail author
  • Yuki Aoki
  • Tomoko Sonoda
  • Miho Yamanishi
  • Ayumu Arakawa
  • Masanaka Sugiyama
  • Nami Shirakawa
  • Sae Ishimaru
  • Yoshimasa Saito
  • Akiko Maeshima
  • Miho Maeda
  • Chitose Ogawa
Case Report
  • 181 Downloads

Abstract

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.

Keywords

Histiocytic sarcoma Rosai–Dorfman disease MAP2K1 MEK inhibitor Trametinib 

Notes

Compliance with ethical standards

Conflict of interest

The authors have no conflict of interests to disclose.

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Copyright information

© The Japanese Society of Hematology 2018

Authors and Affiliations

  • Tadashi Kumamoto
    • 1
    Email author
  • Yuki Aoki
    • 1
  • Tomoko Sonoda
    • 1
  • Miho Yamanishi
    • 4
  • Ayumu Arakawa
    • 1
  • Masanaka Sugiyama
    • 1
  • Nami Shirakawa
    • 1
  • Sae Ishimaru
    • 1
  • Yoshimasa Saito
    • 2
  • Akiko Maeshima
    • 3
  • Miho Maeda
    • 4
  • Chitose Ogawa
    • 1
  1. 1.Department of Pediatric OncologyNational Cancer Center HospitalTokyoJapan
  2. 2.Department of PharmacyNational Cancer Center HospitalTokyoJapan
  3. 3.Department of PathologyNational Cancer HospitalTokyoJapan
  4. 4.Department of PediatricsNippon Medical SchoolTokyoJapan

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