Desmoplastic fibroma: a case report with three years of clinical and radiographic observation and review of the literature

Abstract

Background: Desmoplastic fibroma (DF) is an extremely rare locally aggressive bone tumor with an incidence of 0.11% of all primary bone tumors. The typical clinical presentation is pain and swelling above the affected area. The most common sites of involvement are the mandible and the metaphysis of long bones. Histologically and biologically, desmoplastic fibroma mimics extra-abdominal desmoid tumor of soft tissue.

Case presentation and literature review: A case of a 27-year old man with DF in the ilium, including the clinical, radiological and histological findings over a 4-year period is presented here. CT scans performed in 3-year intervals prior to surgical intervention were compared with respect to tumor extension and cortical breakthrough. The patient was treated with curettage and grafting based on anatomical considerations. Follow-up CT scans over 18-months are also documented here. Additionally, a review and analysis of 271 cases including the presented case with particular emphasis on imaging patterns in MRI and CT as well as treatment modalities and outcomes are presented.

Conclusion: In patients with desmoplastic fibroma, CT is the preferred imaging technique for both the diagnosis of intraosseus tumor extension and assessment of cortical involvement, whereas MRI is favored for the assessment of extraosseus tumor growth and preoperative planning. While tumor resection remains the preferred treatment for DF, curettage and grafting prove to be an acceptable alternative treatment modality with close follow-up when resection is not possible. Curettage and grafting have been shown to provide good clinical results and are associated with long recurrence free intervals.

Keywords: Desmoplastic fibroma; autograft; benign bone tumor; curettage; rare bone tumor; review..

Fig. (1)

CT-scan three years preoperative. The corticalis is preserved. Within the lesion pseudo-trabeculation is visible.

Fig. (2)

T1-weighted MRI three years preoperative. Note that the lesion presents with low signal intensity and no infiltration of the surrounding tissue.

Fig. (3)

CT-scan 2 weeks before the operation. There is cortical breakthrough of the tumor. The size of the lesion has slightly increased in comparison the previous CT-scan.

Fig. (4)

Histological examination of biopsy shows abundant collagen fibers, strong positive labelling for the adhesion protein β-Catenin, weak positive labelling for S-100 and almost no positive stain for the proliferation marker Ki67.

Fig. (5)

Intraoperative photograph showing the removed tissue. It has a beige color and hard as well as soft areas of consistency.

Fig. (6)

CT-scan 6 months after the operation. No residual tumor is present.

Fig. (7)

CT-scan 18 months after the operation. The bone graft is well integrated in pelvic bone. No discontinuity of the cortical bone. No recurrence of the tumor is present.

Fig. (8)

Distribution of desmoplastic fibroma in 266 patients.