Characterization of childhood precursor T-lymphoblastic lymphoma by lmmunophenotyping and fluorescent in situ hybridization: A report from the Children's Oncology Group

Kristi J. Smock, Marilu Nelson, Sheryl R. Tripp, Warren G. Sanger, Minnie Abromowitch, Mitchell S. Cairo, Sherrie L. Perkins

Research output: Contribution to journalArticle

21 Scopus citations


Background. T-lymphoblastic lymphoma (T-LBL) accounts for 25-30% of childhood non-Hodgkin's lymphoma and is closely related to T-lymphoblastic leukemia (T-ALL). Recently, we demonstrated distinct differences in gene expression between childhood T-LBL and T-ALL, but molecular pathogenesis and relevant protein expression patterns in T-LBL remain poorly understood. Procedure. Children with T-LBL with disseminated disease were registered and treated on COG protocol 5971. Paraffin-embedded tumor tissue was obtained at diagnosis for immunohistochemistry (IHC) and fluorescence in situ hybridization (FISH) studies. We determined the pattern and intensity of staining for c-Myc, Skp2, Mib-1, p53, TCL-1, bcl-2, and bcl-6 proteins by IHC and c-Myc, p53, bcl-2, bcl-6, and TCR α/δ molecular alterations by FISH in 22 pediatric T-LBL cases. Results. The majority of T-LBL samples expressed Mib-1 (59%) and c-Myc (77%) proteins in greater than 50% of the cells, but Skp2 (14%), p53 (14%), and bcl-2 (23%) expression was less common. FISH studies demonstrated 18% gains and 10% losses in c-Myc, 16% gains in p53, 12% gains and 6% losses in bcl-2, and 6% gains and 19% losses in bcl-6 with little direct correlation between the IHC and FISH studies. Conclusions. Childhood T-LBL is a highly proliferative tumor associated with enhanced expression of c-Myc protein, but without detectable c-Myc molecular alterations. FISH studies did not identify consistent etiologies of molecular dysregulation, and future studies with other molecular approaches may be required to elucidate the molecular pathogenesis of childhood T-LBL.

Original languageEnglish (US)
Pages (from-to)489-494
Number of pages6
JournalPediatric Blood and Cancer
Issue number4
StatePublished - Oct 1 2008



  • Fluorescence in situ hybridization
  • Immunohistochemistry
  • Immunophenotyping
  • Molecular analysis
  • T-cell lymphoblastic lymphoma

ASJC Scopus subject areas

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

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