Extrapleural solitary fibrous tumor: a report of seven cases

Solitary fibrous tumors (SFTs) are rare, spindle-cell neoplasms generally associated with the serosal surface, especially the pleura. Histopathologic, immunohistochemical, ultrastructural, and flow cytometric analyses were performed on seven SFTs of extrapleural sites (two retroperitoneal, two soft tissue, one each peritoneal, nasal cavity, and orbit). Five patients were women, and two were men, aged from 21 to 68 years (average, 39 yr). All of the lesions presented as well-circumscribed masses. The lesions ranged in size from 2 to 20 cm in greatest diameter. Histologically, these lesions were entirely comparable to the pleural SFTs and lacked the characteristic features of other recognized neoplasms that occur in these regions. One tumor contained pleomorphic and round-cell sarcomatous foci. Immunohistochemically, all of the tumors were strongly positive for vimentin and CD34. Six of the seven tumors showed varying numbers of spindle cells positive for alpha smooth muscle actin, HHF35, neuron-specific enolase, Leu 7, or glial fibrillary acidic protein. Ultrastructural examinations of three tumors showed that they were composed of primitive mesenchymal or fibroblast-like cells. Six tumors examined were diploid by flow cytometric examination. Clinical follow-up in six patients ranged from 1 to 7.5 years (average, 2.6 yr) and showed that five patients remained well with no evidence of disease after excision and that the patient with the sarcomatous elements died of recurrence 2.5 years after surgical treatment. These findings suggest that SFTs represent ubiquitous neoplasms of fibroblasts or primitive mesenchymal cells with aggressive potential.