Pediatric histiocytic sarcoma clonally related to precursor B-cell acute lymphoblastic leukemia with homozygous deletion of CDKN2A encoding p16INK4A.
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 p16(INK4A). 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.
Division of Pediatric Hematology-Oncology, Mayo Clinic, Rochester, Minnesota 55905, USA.
Riten
R
Kumar
Shakila P
SP
Khan
Divya-Devi
DD
Joshi
Gene R
GR
Shaw
Rhett P
RP
Ketterling
Andrew L
AL
Feldman
D002469:Cell Separation; D002675:Child, Preschool; D019941:Cyclin-Dependent Kinase Inhibitor p16 / Q000235:genetics; D005434:Flow Cytometry; D005663:Furans; D019942:Genes, p16; D054747:Histiocytic Sarcoma / Q000235:genetics* / Q000473:pathology; D006801:Humans; D017404:In Situ Hybridization, Fluorescence; D008297:Male; D016609:Neoplasms, Second Primary / Q000235:genetics* / Q000473:pathology; D015452:Precursor B-Cell Lymphoblastic Leukemia-Lymphoma / Q000235:genetics*; D013876:Thiophenes
C516387:3,4-bis-(2,4,5-trimethyl-thiophen-3-yl)furan-2,5-dione; D019941:Cyclin-Dependent Kinase Inhibitor p16; D005663:Furans; D013876:Thiophenes