Pediatric histiocytic sarcoma clonally related to precursor B-cell acute lymphoblastic leukemia with homozygous deletion of CDKN2A encoding p16INK4A

Riten Kumar, Shakila P. Khan, Divya Devi Joshi, Gene R. Shaw, Rhett P. Ketterling, Andrew L Feldman

Research output: Contribution to journalArticle

38 Scopus citations


Histiocytic sarcoma (HS) is a rare malignancy of tissue histiocytes with a dismal prognosis. We report a 4-year-old male who developed HS during maintenance chemotherapy for precursor B-cell acute lymphoblastic leukemia (pre-B ALL). Both tumors showed identical clonal immunoglobulin and T-cell receptor gene re-arrangement patterns, as well as homozygous deletion of the CDKN2A gene encoding p16INK4A. These data suggest a clonal relationship between the two neoplasms despite their distinct lineages. Since CDKN2A deletion predisposes to development of HS in experimental models, the cytogenetic features of the patient's pre-B ALL may have predisposed to this change in lineage.

Original languageEnglish (US)
Pages (from-to)307-310
Number of pages4
JournalPediatric Blood and Cancer
Issue number2
StatePublished - Feb 2011



  • B-cell acute lymphoblastic leukemia
  • CDKN2A
  • Histiocytic sarcoma
  • P16
  • Trans-differentiation

ASJC Scopus subject areas

  • Oncology
  • Pediatrics, Perinatology, and Child Health
  • Hematology

Cite this