Fibrohistiocytic differentiation in subcutaneous fatty tumors. Study of spindle cell, pleomorphic, myxoid, and atypical lipoma and dedifferentiated liposarcoma cases composed in part of CD34+ fibroblasts and FXIIIa+ histiocytes.

Subsets of dendritic cells, fibroblasts which express the human progenitor cell antigen CD34 or histiocytes which express coagulation factor XIIIa (FXIIIa), are present in fat and in collagenous connective tissue. As components of the microvascular unit, these fibrohistiocytic cell subsets may interact during stromal remodeling, repair, and neoplasia. We studied white fat and subcutaneous fatty tumors to determine if CD34 and/or FXIIIa+ "fibrohistiocytic" dendritic cell subsets are involved in their morphogenesis. Three lipomas (L), 1 intramuscular lipoma (IL), 1 myxoid lipoma (ML), 2 pleomorphic lipomas (PL), 2 spindle cell lipomas (SCL), 8 angiolipomas (AN) in 4 patients, 1 atypical lipoma/well-differentiated liposarcoma (AL), 1 de novo dedifferentiated liposarcoma (DL), and 1 recurring atypical myxoid signet ring lipomatous tumor were examined for CD34, FXIIIa and in some cases for CD31, desmin, Ki 67, or S-100. Normal fat has scattered CD34+ dendritic cells and small FXIIIa+ dendritic histiocytes among variably S-100+ adipocytes. The CD34 and FXIIIa+ dendritic cells are more numerous near vessels and within fibrovascular septae. In L and IL, CD34 and FXIIIa+ dendritic cells are activated and some adipocytes express CD34. Mesenchymal areas of SCL, PL, ML, and AL and DL are composed of CD34+ dendritic cells with CD34+ but FXIIIa-negative floret cells in PL or atypical cells in AL and DL. FXIIIa+ dendritic cells are numerous in these lesions, comprising 30-40% of cells in SCL and PL, and 50% in ML, AL, and DL. AN have focal CD34+ interstitial cells and plump FXIIIa+ cells that in one case resembled multivacuolated lipoblasts. The myxoid signet ring lipomatous tumor was CD34 negative with few FXIIIa+ cells. We conclude that subsets of CD34+ and FXIIIa+ dendritic microvascular cells are present in normal fat and proliferate together in various types of lipomas and in at least some dedifferentiated liposarcomas.