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.

Desmoplastic fibroma in the proximal femur: A case report with long-term follow-up

“…Desmoplastic fibroma of the bone is an extremely rare bone tumor that resembles a desmoid tumor of the soft tissues. In 1958, Jaffe (7) first differentiated this bony lesion from other fibromas of bone, and termed it 'desmoplastic fibroma' based on the presence of fibroblasts and abundant collagenous tissue (21).…”

“…Aggressive fibromatosis, also known as a desmoid tumor, was described prior to desmoplastic fibroma and is defined as a rare mesenchymal neoplasia composed of collagenous tissue and spindle-shaped cells (7). The majority of desmoid tumors are abdominal (69%); they can be localized intra-abdominally, within the abdominal wall and also extra-abdominally (7,21). As the intraosseus variant is desmoplastic fibroma, morphological differentiation is unlikely.…”

“…The cortex is usually thin and there are intralesional trabeculae, resembling soap bubbles. Computed tomography is used to detect intraosseus tumor extension and cortical involvement, while magnetic resonance imaging demonstrates the separation of the intraosseus tumor from the bone, and is used to assess extraosseus tumor growth and for pre-operative planning (1,2,21). The radiologic differential diagnosis includes non-ossifying fibroma, fibrous dysplasia, adamantinoma, bone cysts, giant cell tumors and metastases.…”

“…When a resection is feasible, as in the mandible and long bones, it should be performed, as a resection has the lowest rate of recurrence (1). Nedopil et al (21) suggested that in isolated intraosseus lesions without evidence of extension in adjacent soft tissus, and when resection is associated with a higher risk due to anatomical conditions, a risk-benefit analysis should be performed. In these cases, thorough curettage may be adequate management, as it reduces the surgical duration, has a lower risk of infection and facilitates a faster recovery.…”

Ultrasonography of desmoplatic fibroma of the mandible; a case report.

“…It is considered to be a very aggressive tumor with a local recurrence up to 67% of the cases, after surgical resection [1]. This remarkable rare tumor (0.11% of all primary bone tumors) is histological similar to the desmoid tumor with extra-abdominal distribution [1,2]. Between 1968 and 2011 only 18 cases of DF of the mandible, in patients younger than 4 years-old were reported [3].…”