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

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.

• Authors: Tadashi Kumamoto, Yuki Aoki, Tomoko Sonoda, Miho Yamanishi, Ayumu Arakawa, Masanaka Sugiyama, Nami Shirakawa, Sae Ishimaru, Yoshimasa Saito, Akiko Maeshima, Miho Maeda, Chitose Ogawa
• Journal: International journal of hematology (Int J Hematol) Vol. 109 Issue 2 Pg. 228-232 (Feb 2019) ISSN: 1865-3774 [Electronic] Japan
• PMID: 30361829 (Publication Type: Case Reports, Journal Article)

Chemical References

• Protein Kinase Inhibitors
• Pyridones
• Pyrimidinones
• trametinib
• MAP Kinase Kinase 1
• MAP2K1 protein, human

Topics

• Adolescent
• Histiocytic Sarcoma (drug therapy)
• Humans
• MAP Kinase Kinase 1 (genetics)
• MAP Kinase Signaling System (drug effects)
• Male
• Protein Kinase Inhibitors (therapeutic use)
• Pyridones (therapeutic use)
• Pyrimidinones (therapeutic use)
• Recurrence
• Treatment Outcome