Collagenous fibroma (desmoplastic fibroblastoma): A clinicopathologic analysis of 63 cases of a distinctive soft tissue lesion with stellate-shaped fibroblasts

Abstract

Sixty-three cases of collagenous fibroma (desmoplastic fibroblastoma) from the files of the Armed Forces Institute of Pathology were analyzed. These tumors occurred mostly in men (80%) with a median age of 50 years (range, 16 to 81 years). The lesions had a wide anatomic distribution and involved the arm (24%), shoulder girdle (19%), posterior neck or upper back (14%), feet or ankles (14%), leg (14%), hand (8%), and abdominal wall and hip (6%). The patients typically presented with a history of a painless, slowly growing mass, often of relatively long duration. The tumors ranged in size from 1 to 20 cm (median, 3.0 cm). The lesions were predominantly subcutaneous, but fascial involvement was common, and 27% of cases involved skeletal muscle. Gross examination typically showed an elongated, lobulated, or disc-shaped mass with a firm consistency and a homogeneous pearl-gray color. Histologically, the tumors often appeared well marginated on low-power examination, but most (78%) infiltrated fat or, less commonly, skeletal muscle. The lesional cells were relatively bland stellate and spindle-shaped fibroblasts separated by a collag enous or myxocollagenous matrix. Mitotic activity was absent or minimal. Some of the lesional cells had a myofibroblastic immunophenotype, as evidenced by focal reactivity for muscle-specific and a-smooth muscle actins. In a few cases, rare actin-positive cells were also positive for keratins. Desmin, S100 protein, and CD34 were not expressed. None of the 39 patients with follow-up (median, 11 years) developed a recurrence. Collagenous fibroma is a benign fibroblastic/ myofibroblastic proliferation. The large size of some of these tumors coupled with slow growth and persistence favors a neoplastic process over a peculiar reactive proliferation. The differential diagnosis includes a variety of reactive and neoplastic fibroblastic lesions, most importantly fibromatosis and low-grade fibromyxoid sarcoma. Simple, conservative excision is the treatment of choice for collagenous fibroma.