Deep "benign" fibrous histiocytoma: clinicopathologic analysis of 69 cases of a rare tumor indicating occasional metastatic potential.

Benign fibrous histiocytoma (FH) is one of the most common mesenchymal neoplasms of the skin. Several histologic variants of cutaneous FH have been described, some of which also have distinct clinical features including a propensity for local recurrence. Deep benign FH is an uncommon and poorly recognized clinical subtype that arises in subcutaneous or deep soft tissue. Only a single small series of these neoplasms has been published, and their clinical behavior is not well characterized. In this study, we report the clinicopathologic features of 69 deep FH retrieved from our consultation files. The patients included 41 males and 28 females, ranging in age from 6 to 84 years (median, 37 y). The most common anatomic location was the extremities (58%); the remainder arose on the head and neck (22%), trunk (11%), and in the deep soft tissue of the retroperitoneum, mediastinum, or pelvis (9%). All lesions arising in nonvisceral soft tissue were subcutaneous. The tumors ranged from 0.5 to 25 cm in size (median, 3.0 cm) and were well circumscribed grossly and microscopically. All tumors were composed of bland ovoid to spindle cells arranged in a storiform pattern with admixed lymphocytes. Multinucleate giant cells, osteoclastic giant cells, and/or foam cells were present in 59% of cases, whereas the other 41% were cytologically monomorphic, often resembling cellular FH. Other common findings included a hemangiopericytomalike vascular pattern (42%) and stromal hyalinization (39%). Four cases were classified as atypical deep FH due to the presence of scattered markedly pleomorphic spindle cells within an otherwise histologically typical lesion. The median mitotic rate was 3/10 HPF; 10 cases (14%) had >10 mitoses/10 HPF. Necrosis (2 cases) and lymphovascular invasion (1 case) were rare. Immunohistochemistry revealed expression of CD34 in 20/50 cases (40%), smooth muscle actin in 15/40 (38%), and focal desmin in 1/12 (8%). Of the 37 patients for whom clinical follow-up was available (median, 40 mo), 8 (22%) had a local recurrence; in all 8 cases, the tumor had been marginally or incompletely excised. Metastases occurred in 2 patients (5%), both of whom ultimately died of disease; however, this number is likely exaggerated due to consultation bias. The metastasizing tumors were large (6 and 9 cm) and 1 had tumor necrosis but they were otherwise histologically identical to the nonmetastasizing lesions. In summary, deep FH has many histologic features in common with cutaneous cellular FH; however, it usually has a more diffusely storiform pattern than the latter, is well circumscribed, and may have striking hemangiopericytomalike vessels. Similar to the cellular, aneurysmal, and atypical variants of FH, deep FH recurs in approximately 20% of cases and may rarely metastasize.