Desmoplastic fibroma of bone: a report of 18 cases

Abstract

Desmoplastic fibroma is a rare benign primary bone tumor, histologically identical to the extra-abdominal desmoid tumor of soft tissues. The incidence in major series of bone tumors is 0.1%-0.3%. In the present study 18 cases from the files of the Netherlands Committee on Bone Tumors are reported, with an emphasis on the radiographic features. Eight female and ten male subjects were affected, with a fairly even age distribution from the second to the seventh decade of life. The most frequent sites of involvement were the femur and the pelvis. Radiographically the lesions nearly always demonstrated benign features with respect to pattern of destruction, margins, and reactive bone formation; however, soft tissue extension was sometimes present. Although desmoplastic fibroma usually presents with pain or swelling, in 5 of the 18 cases the existence of the lesion was an accidental finding, and in 2 cases the patient had long-standing, vague complaints of pain. Though overall benign radiographic features may support the final diagnosis, the main and often troublesome histological differential diagnosis is of a low-grade fibrosoma. En bloc resection is the treatment of choice in view of the high incidence of recurrence after curettage.

Desmoplastic fibroma of the spine causing severe mediastinal compression and brachial plexus encasement

“…16,20 Classically, DFs have been described as slow growing and relatively benign. 3,24,28 However, this description is not consistent with the DFs we encountered in our two patients; those tumors caused bony destruction and mediastinal compression leading to hypotension over a relatively short period of time. New genetic studies have shown that DFs have the potential to develop malignant transformation characteristics.…”

“…22 Although DF accounts for only 0.1%-0.3% of all bone tumors and is typically characterized as a slow-growing benign lesion, 28 it can be locally aggressive and cause destruction of bone and soft tissue. 11 In addition, DF is notorious for local recurrence even after resection 10 and, on rare occasions, can grow quickly, causing significant morbidity.…”

“…11 In addition, DF is notorious for local recurrence even after resection 10 and, on rare occasions, can grow quickly, causing significant morbidity. Patients most commonly present in the 2nd or 3rd decades of life without gender bias, 28 with disease appearing in the long tubular bones, such as the femur and humerus, and very rarely in the pelvis, skull, or spine. 5 To our knowledge, there have been only 4 reports of DF involving the cervical spine, 1,7,28,30 5 cases of thoracic DF, 9,12,15,21,27 2 cases of lumbar DF, 13,25 and 2 cases of sacral DF 24,31 (Table 1).…”

Incidental lesion in the femoral metaphysis

“…The treatment of choice, therefore, is wide tumor resection. Taconis et al in their analysis of 18 cases of desmoplastic tumor, found no recurrence occurring after 9-year follow-up in patients treated with wide resection [2].…”

“…Clinically, the commonest presentation is pain, palpable mass, and swelling; although it may be discovered as incidental finding on imaging. The lesion may also become evident after a pathological fracture [2]. The majority of cases are reported in patients younger than 30 years of age.…”