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4 June 2014 - Case #314

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Thanks to Dr. Weiwei Chen, State University of New York (SUNY) at Buffalo (USA), for contributing this case.


   

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Case #314

Clinical history:
A 68 year old woman presented with a history of CLL and papillary thyroid carcinoma. She was recently found to have progressively enlarging right inguinal lymph nodes. One of the lymph nodes was biopsied.

Microscopic images:




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Diagnosis: Synchronous Langerhans cell sarcoma and small lymphocytic lymphoma / chronic lymphocytic leukemia

Immunostains:

CD1a

CD5

CD20

CD23

Langerin

S100



Discussion:
Langerhans cell sarcoma is a rare tumor with markedly malignant cytological features that originates from Langerhans cells (IARC: WHO Classification of Tumours of Haematopoietic and Lymphoid Tissue, 4th Edition 2008). This case appears to be an example of transdifferentiation, first described by Jaffe et al. in which histiocytic / dendritic cell sarcomas may be transformed from lymphoproliferative diseases (N Am J Med Sci 2013;5:386, Blood 2008;111:5433, Am J Surg Pathol 2013;37:978). In this case, both the Langerhans cell sarcoma and CLL are clonally related, and contain the BRAF V600E mutation.

Langerhans cell sarcoma (LCS) is often an extranodal tumor with skin and bone involvement but it may present with multiorgan involvement including lymph node, lung, liver and spleen. As with Langerhans cell histiocytosis (LCH), it is a clonal proliferation of Langerhans cells, which are 12 - 15 microns in diameter with abundant, pale eosinophilic cytoplasm, irregular and elongated nuclei with prominent nuclear grooves and folds, fine chromatin and indistinct nucleoli. Langerhans cells are immunoreactive to CD1a, langerin and S100. However, LCS compared with LCH has more cytological atypia, a higher proliferation rate and a more aggressive clinical course (Diagn Pathol 2012;7:128).


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