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Open Access Case Report

Langerhans cell sarcoma with an aberrant cytoplasmic CD3 expression

Zhaodong Xu1*, Ruth Padmore1, Carolyn Faught2, Lisa Duffet2 and Bruce F Burns3

Author Affiliations

1 Department of Pathology and Laboratory Medicine, Division of Hematopathology, The Ottawa Hospital, 501 Smyth Road, Ottawa, Ontario, Canada

2 Department of Medicine, Division of Hematology, The Ottawa Hospital, 501 Smyth Road, Ottawa, Ontario, Canada

3 Department of Pathology and Laboratory Medicine, Division of Anatomical Pathology, The Ottawa Hospital, 501 Smyth Road, Ottawa, Ontario, Canada

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Diagnostic Pathology 2012, 7:128  doi:10.1186/1746-1596-7-128

Published: 25 September 2012

Abstract

Langerhans cell sarcoma is a rare and aggressive high grade hematopoietic neoplasm with a dismal prognosis. It has a unique morphological and immunotypic profile with a CD1a/ langerin/S100 + phenotype. T cell lineage markers except for CD4 in Langerhans cell sarcoma have not been documented previously. We report a case of 86 year-old male of Caucasian descent who presented with an enlarging right neck mass over 2 months with an underlying unknown cause of anemia. Computed tomography scan of the neck, chest and abdomen revealed generalized lymphadenopathy and mild splenomegaly suspicious for lymphoma. Diagnostic core biopsy performed on right neck mass revealed a possible T cell lymphoma with expression of T cell lineage specific marker CD3 but conclusive diagnosis could not be made due to insufficient core biopsy sample. Further excisional biopsy performed on a left inguinal node showed a hematopoietic neoplasm with features of Langerhans cell sarcoma with a focal cytoplasmic CD3 expression in 30-40% of the tumor cells. PCR for T cell receptor (TCR) gene rearrangement failed to demonstrate a clonal gene rearrangement in the tumor cells arguing against a T cell lineage transdifferentiation, suggesting an aberrant CD3 expression. To the best of our knowledge, this case represents the first report of Langerhans cell sarcoma with an aberrant cytoplasmic CD3 expression.

http://www.diagnosticpathology.diagnomx.eu/vs/2065486371761991 webcite

Keywords:
Langerhans cell sarcoma (LCS); Langerhans cell histiocytosis (LCH); CD3; Aberrant expression; Lineage plasticity; Transdifferentiation