Primary central nervous system histiocytic sarcoma arising after precursor B-cell acute lymphoblastic leukemia

Alan F. Brown, Hongxin Fan, John R. Floyd, James M Henry, Russell A. Higgins

Research output: Contribution to journalReview articlepeer-review

12 Scopus citations


Histiocytic sarcomas (HSs) are rare malignant neoplasms derived from histiocytes that may be associated with other hematolymphoid neoplasms. Histiocytic sarcomas rarely occur in the CNS and have not previously been reported in conjunction with prior B-cell lymphoblastic leukemia. We report the case of a 23-year-old man who presented with primary CNS HS 7 years after achieving remission for precursor B-cell acute lymphoblastic leukemia (B-ALL). Molecular studies revealed clonal immunoglobulin heavy-chain (IGH) gene rearrangement within the HS, suggesting linkage to his previous B-ALL. Previously reported post-ALL HSs show a strong predilection for young males (male-to-female ratio, 20:1), whereas cases of primary CNS HS without previous ALL affected older adults with balanced sex predilection. The patient's survival at 60 months exceeds expectations when compared with that of other reported cases of de novo primary CNS HS (n = 18) and post-ALL HS at all sites (n = 19). In addition, we discuss the potential relationship between B-ALL and HS posed by other authors.

Original languageEnglish (US)
Pages (from-to)1120-1126
Number of pages7
JournalJournal of Neuropathology and Experimental Neurology
Issue number12
StatePublished - 2015


  • Acute lymphoblastic leukemia
  • Central nervous system
  • Clonality
  • Delayed recurrence
  • Histiocytic sarcoma
  • Transdifferentiation

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Neurology
  • Clinical Neurology
  • Cellular and Molecular Neuroscience


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