Pleomorphic hyalinizing angiectatic tumor of soft parts: Immunohistochemical case study shows cellular composition by CD34+ fibroblasts and factor XIIIa+ dendrophages

We report immunomorphologic observations on a pleomorphic hyalinizing angiectatic tumor of soft parts (PHAT), a rare tumor recently described by Smith, Fisher, and Weiss. A 2 cm skin-covered, grossly lobulated, firm, yellow-tan, focally hemorrhagic tumor was excised from the dorsum of a 59-year-old woman's right foot. It infiltrated dermis and subcutis and entrapped skin adnexae. The tumor microscopically resembled both a pleomorphic malignant fibrous histiocytoma and a neurilemoma with fascicular spindle cell pattern, pleomorphic tumor giant cells, and focal congeries of ectatic, fibrinous, and slightly hyalinized vessels. Tumor cells produced abundant reticulin but collagenous sclerosis was minimal. Mast cells were numerous. Pleomorphic cells, some phagocytic, had intranuclear vacuolar inclusions and many cells had large pale cytoplasmic globular inclusions. Most tumor cells expressed vimentin and CD34, including pleomorphic cells. Factor XIIIa stained focally 20-40% of the spindle cells. S100 and cytokeratin were negative and actin and desmin stained only vessel myopericytes. The Ki 67 index was 3% with mostly large CD34+ cells and a few smaller FXIIIa+ cells in the cycling fraction. We conclude that PHAT is a fibrohistiocytic tumor probably derived from proliferating microvascular CD34+ dendritic cells and FXIIIa+ dendrophage cell subsets. Possible interactions between these cell types deserve further study in PHAT and other fibrohistiocytic tumors. (C) Munksgaard 1997.