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4 June 2014 - Case of the Week #314

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Case of the Week #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.

Micro images:

What is your diagnosis?

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

Immunostains were obtained:







Langerhans cell sarcoma is a rare tumor with markedly malignant cytological features that originates from Langerhans cells (WHO Classification of Tumours of Haematopoietic and Lymphoid Tissue, IARC, 2008). This case, also described at N Am J Med Sci 2013;5:386, appears to be an example of transdifferentiation, first described by Jaffe et. al. (Blood 2008;111:5433), in which histiocytic/dendritic cell sarcomas may be transformed from lymphoproliferative diseases (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 multi-organ 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 to LCH has more cytological atypia, a higher proliferation rate and a more aggressive clinical course (Diagn Pathol 2012;7:128).

Nat Pernick, M.D., President
and Shivani Thakore, Associate Medical Editor
PathologyOutlines.com, Inc.
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Bingham Farms, Michigan (USA) 48025
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Email: NatPernick@Hotmail.com
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