Reticulohistiocytoma pathology

Introduction

Reticulohistiocytoma is a non-Langerhans cell histiocytosis. Lesions may occur in isolation (reticulohistocytoma) or in multiplicity (multicentric reticulohistiocytosis). Multicentric reticulohistocytosis is characterized by cutaneous or mucosal papular lesions often associated with severe polyarthritis and arthralgias. Associated paraproteinemia has also been described.

Histology of reticulohistiocytoma

In reticulohistiocytoma, sections show a diffuse infiltration of numerous large, mononucleated or multinucleated histiocytes in the dermis (figures 1-3). There are associated lymphocytes and dermal fibrosis. Characteristically, the lesional cells have a dense pink cytoplasm, variously referred to as “oncocytic” or “ground glass” (figures 2, 3).

Figure 1

Figure 2

Figure 3

Special studies for reticulohistiocytoma

Reticulohistiocytoma cells are positive with CD68 and Factor 13a. S100 and CD1a are typically negative.

Differential diagnosis of reticulohistiocytoma

Other histiocytic diseases: these typically lack the characteristic “ground glass” cytoplasm seen in reticulohistiocytoma. In addition to morphologic features, CD1a and S100 immunohistochemical studies can be used to exclude Langerhans cell histiocytosis and Rosai-Dorfman disease respectively.

Juvenile xanthogranuloma shows “Touton” giant cells, and lipidisation of histiocytes. The rarer histocytoses may require more extensive immunohistochemical studies and clinical correlation.