Infantile subconjunctival and anterior orbital fibrous histiocytoma. Ultrastructural and immunohistochemical studies

A fleshy, polypoidal and partially lobulated lesion that protruded between the eyelids from the medial caruncular region and that infiltrated the contiguous anterior orbital tissues developed over 1 month in a 9-month-old infant. The microscopic features of the tumor included a plump spindle cell population, more polygonal cells, early xanthoma cell transformation, infiltrating lymphocytes and eosinophils, and multinucleated giant cells, the last not exhibiting classic Touton characteristics. The histopathologic differential diagnosis ranged among fibrous histiocytoma, juvenile xanthogranuloma, and eosinophilic granuloma (histiocytosis-X). Results of electron microscopy disclosed abundant rough-surfaced endoplasmic reticulum, a paucity of lysosomes, and no Langerhans' (Birbeck) granules. Immunohistochemistry corroborated the fibrohistiocytic nature of the tumor, because histochemical stains for the enzymes alpha-1-antichymotrypsin and lysozyme, and monoclonal or polyclonal antibodies against common leukocytic antigen and S-100 protein, were negative--whereas they would have been expected to be positive in various combinations in the different histiocytic proliferations. Vimentin was identified in the tumor cells; this is an intermediate cytoplasmic filament almost always present in mesenchymal proliferations. The distinctions between fibrous histiocytomas of stromal cell origin and true histiocytic proliferations of bone marrow cell provenance are explored.