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Live-3D-Cell Immunocytochemistry Assays of Pediatric Diffuse Midline Glioma

Live-3D-Cell Immunocytochemistry Assays of Pediatric Diffuse Midline Glioma

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Langerhans cell sarcoma with an aberrant cytoplasmic CD3 expression.

Zhaodong Xu¹, Ruth Padmore, Carolyn Faught

¹Department of Pathology and Laboratory Medicine, Division of Hematopathology, The Ottawa Hospital, Ottawa, Ontario, Canada. zhaodong_xu@hotmail.com

Diagnostic Pathology

|September 26, 2012

Summary

This case study reports the first instance of Langerhans cell sarcoma exhibiting aberrant cytoplasmic CD3 expression. This rare hematopoietic neoplasm typically presents with a distinct immunophenotype, making this finding significant for diagnostic pathology.

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Area of Science:

Hematopathology
Oncology
Immunophenotyping

Background:

Langerhans cell sarcoma (LCS) is a rare, aggressive hematopoietic neoplasm with a poor prognosis.
LCS is characterized by a specific immunophenotype: CD1a, langerin, and S100 positive.
T cell lineage markers, apart from CD4, are not typically observed in LCS.

Observation:

A case of an 86-year-old male presenting with a neck mass and anemia.
Initial imaging suggested lymphoma due to generalized lymphadenopathy and splenomegaly.
Biopsies revealed a hematopoietic neoplasm with features of LCS, unexpectedly showing focal cytoplasmic CD3 expression.

Findings:

The tumor cells in this LCS case demonstrated focal cytoplasmic CD3 expression.
Polymerase Chain Reaction (PCR) for T cell receptor (TCR) gene rearrangement was negative, ruling out T cell lineage.

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Live-3D-Cell Immunocytochemistry Assays of Pediatric Diffuse Midline Glioma

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This suggests an aberrant expression of CD3 rather than a true T cell origin.

Implications:

This is the first reported case of Langerhans cell sarcoma with aberrant cytoplasmic CD3 expression.
The finding expands the known immunophenotypic spectrum of LCS.
This case highlights the importance of comprehensive immunophenotypic analysis for accurate diagnosis of rare neoplasms.