%0 Journal Article
%T Langerhans cell sarcoma with an aberrant cytoplasmic CD3 expression
%A Zhaodong Xu
%A Ruth Padmore
%A Carolyn Faught
%A Lisa Duffet
%A Bruce F Burns
%J Diagnostic Pathology
%D 2012
%I BioMed Central
%R 10.1186/1746-1596-7-128
%X http://www.diagnosticpathology.diagnomx.eu/vs/2065486371761991 webciteAccording to the most recent WHO Classification of Tumors of Hematopoietic and Lymphoid Tissues (2008) [1], Langerhans cell sarcoma (LCS) belongs to the category of histiocytic and dendritic cell neoplasms. It is a rare subtype of tumors derived from Langerhans cells with a female predominance.Clinically, LCS is often an extranodal tumor with skin and bone involvement, but it may present with multi-organ involvement including lymph node, lung, liver and spleen. 11% of the reported cases patients had pancytopenia. Compared to more commonly known Langerhans cell histiocytosis (LCH), also a clonal neoplastic proliferation of Langerhans cells, LCS has a much higher degree of cytological atypia and prominent proliferation rate with an aggressive clinical course. Both LCH and LCS have a similar immunophenotype including CD1a/langerin/S100+ [2,3] but without B- and T-cell lineage markers except for CD4 [4]. LCS with a cytoplasmic T cell specific marker CD3 has heretofore not been reported in the English literature. Herein we provide the first report of LCS with an aberrant cytoplasmic CD3 expression.An 86£¿year old Caucasian male who had been relatively healthy with medically controlled diabetes on insulin, treated temporal arteritis and myocardial infarction in 1987 without ongoing angina was referred to hematology/Oncology service for possible T cell lymphoma at Ottawa Hospital in July 2011. The patient had a short history of a rapidly growing right neck mass, an anemia of unknown cause, mild weight loss and generalized weakness. Laboratory investigations showed a bicytopenia with a normocytic anemia, Hb 89£¿g/L (reference interval 115¨C155£¿g/L) and thrombocytopenia, PLT 86 x 109/L (reference interval 125¨C400 x 109/L). He also had a neutrophilia with WBC 19.8 x 109/L (reference interval 3.0-10.5 x 109/L). LDH was mildly elevated with a value of 240 u/L(reference interval 100¨C205 u/L). CT of neck/thorax/ab
%K Langerhans cell sarcoma (LCS)
%K Langerhans cell histiocytosis (LCH)
%K CD3
%K Aberrant expression
%K Lineage plasticity
%K Transdifferentiation
%U http://www.diagnosticpathology.org/content/7/1/128