Differentiating lymphoblastic lymphoma and Ewing's sarcoma: Lymphocyte markers and gene rearrangement

Metin Ozdemirli, Julie Fanburg-Smith, Dan Paul Hartmann, Norio Azumi, Markku Miettinen

Research output: Contribution to journalArticle

62 Citations (Scopus)

Abstract

We encountered a child with an intraosseous small round cell tumor that was negative for LCA, CD20 (L26), and CD3 and positive for vimentin, CD99 (MIC-2), and periodic acid-Schiff. The tumor exhibited rosette-like formations. This case was initially interpreted as Ewing's sarcoma (ES); however, additional studies revealed positivity for CD79a, CD43, and TdT expression, and an immunoglobulin heavy chain gene rearrangement (IgH-R) by polymerase chain reaction (PCR) established this to be a precursor B-lymphoblastic lymphoma. Because the differential diagnosis of ES and lymphoblastic lymphoma can be difficult and the differential diagnostic value of leukocyte antigens and immunoglobulin heavy chain gene rearrangement studies have not been fully evaluated, we conducted a more extensive investigation on 33 (21 soft tissue and 12 intraosseous) ES cases. Cases were retrieved from the files of the Department of Pathology at Georgetown University and from the Soft Tissue Registry of the Armed Forces Institute of Pathology. The cases were studied by light microscopy, immunohistochemistry, and PCR for IgH-R and T cell receptor gamma chain gene rearrangement (Tγ-R). There were 17 females and 16 males; the mean age was 29.3 years. Locations included the extremities (n = 17) and trunk (n = 16). All cases fit the ES spectrum by light microscopy and immunohistochemistry, as previously determined, and were negative for lymphoid markers (LCA, CD3, CD20, CD43, CD79a, and TdT) CD10 and CD34. CD99 was positive in 31/33 and bcl-2 was weakly positive in 13/33 cases. All 21 cases studied for gene rearrangements by PCR were negative for IgH-R and Tγ-R. Distinction of intraosseous lymphoblastic lymphoma from ES may be difficult because lymphomas may occasionally exhibit unexpected morphologic and immunophenotypic properties including LCA, CD3 and CD20 negativity and cytokeratin positivity. Additional analysis using CD79a, CD43, TdT, and PCR should be performed to avoid misdiagnosis. True ES is negative for lymphoid markers including CD79a, CD43, and TdT, as well as for IgH-R and Tγ-R.

Original languageEnglish (US)
Pages (from-to)1175-1182
Number of pages8
JournalModern Pathology
Volume14
Issue number11
DOIs
StatePublished - Nov 26 2001

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Ewing's Sarcoma
Gene Rearrangement
Precursor Cell Lymphoblastic Leukemia-Lymphoma
Lymphocytes
Immunoglobulin Heavy Chain Genes
Polymerase Chain Reaction
gamma-Chain T-Cell Antigen Receptor Gene Rearrangement
Microscopy
T-Cell Receptor gamma Genes
Immunohistochemistry
Pathology
Rosette Formation
Light
Periodic Acid
Vimentin
HLA Antigens
Keratins
Diagnostic Errors
Registries
Lymphoma

All Science Journal Classification (ASJC) codes

  • Pathology and Forensic Medicine

Cite this

Ozdemirli, Metin ; Fanburg-Smith, Julie ; Hartmann, Dan Paul ; Azumi, Norio ; Miettinen, Markku. / Differentiating lymphoblastic lymphoma and Ewing's sarcoma : Lymphocyte markers and gene rearrangement. In: Modern Pathology. 2001 ; Vol. 14, No. 11. pp. 1175-1182.
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Differentiating lymphoblastic lymphoma and Ewing's sarcoma : Lymphocyte markers and gene rearrangement. / Ozdemirli, Metin; Fanburg-Smith, Julie; Hartmann, Dan Paul; Azumi, Norio; Miettinen, Markku.

In: Modern Pathology, Vol. 14, No. 11, 26.11.2001, p. 1175-1182.

Research output: Contribution to journalArticle

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T2 - Lymphocyte markers and gene rearrangement

AU - Ozdemirli, Metin

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