We report two cases of fibrous histiocytoma (FH) associated with discrete nodular aggregates of Langerhans cells (LCs) resembling Langerhans cell histiocytosis (LCH). In addition, the LCs showed positivity for BRAF V600E immunohistochemistry, a finding reported in neoplastic but not reactive LCs. To our knowledge, these cases represent the first published association of FH and LCH.A 71‐year‐old otherwise healthy male had a 10‐mm papule on his upper back excised. The histology (Figure ) showed a well‐circumscribed dermal tumour associated with overlying epidermal hyperplasia. The lesion was composed of histiocytic‐like and myofibroblastic cells arranged in a haphazard manner with a background of scattered mixed inflammatory cells. Haemorrhagic areas, with haemosiderin deposition and focal aneurysmal change, were seen centrally. The features were those of FH of conventional and focally aneurysmal subtype. However, a striking feature about this lesion was the presence of intratumoural aggregates of histiocytoid cells containing coffee bean‐shaped nuclei with a central nuclear groove. These cells were associated with scattered eosinophils and showed focal extension into the epidermis, with smaller aggregates also noted in the adjacent dermis. Immunohistochemistry showed the cells to be positive for S100, CD1a and Langerin, indicating LCs. Immunohistochemistry for BRAF V600E was also positive in the
Histopathology – Wiley
Published: Jan 1, 2018
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