Langerhans cell sarcoma (LCS) is a rare malignancy requiring differential diagnosis from other high-grade nonhematologic and hematologic tumors. The pathogenesis of LCS remains unknown. Notably, LCS and its benign counterpart, Langerhans cell histiocytosis (LCH), are frequently associated with other malignancies. To the best of our knowledge, we describe the first case of LCS in a chronic myelogenous leukemia (CML) patient undergoing imatinib mesylate therapy. We performed molecular cytogenetic analyses for investigating the association between LCS and CML. In our case, molecular cytogenetic analysis did not reveal BCR-ABL1 fusion and BRAF V600E mutation, suggesting that LCS may be coincident in this patient. However, recurrent BRAF V600E mutation has been found in LCH. Published reports have revealed the clonal relationship between LCH/LCS and other hematologic malignancies, especially lymphoid neoplasms. However, there are only 2 reports demonstrating the clonal relationship between LCH and myeloid neoplasms. The association of LCH/LCS with myeloid neoplasms and the role of BRAF V600E mutation in LCS are discussed.
Keywords: BCR-ABL; BRAF; Langerhans cell sarcoma; chronic myelogenous leukemia; langerin.
© The Author(s) 2013.