Angiofibroma of soft tissue: clinicopathologic characterization of a distinctive benign fibrovascular neoplasm in a series of 37 cases.

Thirty-seven cases of a distinctive benign fibrovascular soft tissue tumor that may be mistaken for a low-grade sarcoma are described. There were 25 female and 12 male patients, ranging in age from 6 to 86 years (median, 49 y). The tumors presented most commonly as a slowly growing painless mass located in the soft tissues of the extremities, mainly the lower extremity, often in relationship to joints or fibrotendinous structures. Most lesions (29 cases) were well circumscribed, ranging in size from 1.2 to 12 cm (median, 3.5 cm). The microscopic appearance was remarkably consistent and was characterized by 2 components: a relatively uniform proliferation of bland, spindle-shaped cells with inconspicuous cytoplasm and ovoid-to-tapering nuclei set in a variably collagenous or myxoid stroma and a prominent vascular network composed of numerous small, branching, thin-walled blood vessels, often accompanied by medium-sized round or irregular and ectatic vessels. Mitoses (1-4/10 hpf) were occasionally observed (9 cases). Mild degenerative nuclear atypia was uncommon (5 cases). Tumor cells expressed epithelial membrane antigen at least focally in 16 of 36 cases (44%), CD34 and smooth muscle actin in 5 cases (14%), and desmin in 4 cases (11%); none expressed S100 protein. Five out of 6 cases analyzed cytogenetically showed a simple karyotype with a balanced t(5;8) chromosomal translocation. Treatment consisted of surgical resection: either simple excision (29 cases), wide excision (6 cases), or amputation (1 case). Follow-up information was available for 28 patients (range, 6 to 144 mo; mean, 51.9 mo). Most patients were alive with no evidence of disease, regardless of the status of surgical resection margins. Four patients developed local recurrence 9, 13, 36, and 120 months after the primary tumor was removed; only in 1 case was there an association with extensively positive surgical resection margins. One of these patients developed a second recurrence 2 months after the first one. None of the patients developed metastasis. The designation "angiofibroma of soft tissue" is proposed to reflect both the likely fibroblastic nature of the proliferating cells and the prominent vascularization of this benign soft tissue neoplasm.