Desmoplastic fibroma of the femur with atypical image findings

Abstract

Rationale:Desmoplastic fibroma of the bone (DFB) is an extremely rare benign bone tumor, which can occur in any bone at all ages. Herein, we report a case of non-specific imaging findings.Patient concerns:A 25-year-old female patient was consulted in the Armed Police General Hospital (Beijing, China) due to repeated pain in the right thigh lasting over 1 year. Imaging examination revealed a space-occupying lesion in the right femur.Diagnosis:Desmoplastic fibroma of the femurInterventions:Wide surgical resectionOutcomes:At the 1-year follow-up visit, no relapse in the tumor was observed.Lessons:In this case report, we described and emphasized the particularity of the case examined and the possible non-specificity of the imaging results of some DFB cases.

Distal Femoral Replacement as a Salvage Treatment After Desmoplastic Fibroma Resection

“…Their reported incidence is about 2.5 patients per 100 million people per year [ 6 ]. Its clinical manifestations usually include pain and swelling in the affected region and symptoms produced by mass compression on the surrounding tissue [ 2 ]. Other patients may debut with a pathologic fracture.…”

“…Other patients may debut with a pathologic fracture. Radiologically, these tumors are centrally located, expansile osteolytic lesions with honeycombed appearance secondary to an irregular trabecular formation through the lytic regions [ 2 , 11 , 12 ]. Their diagnosis is challenging on radiographical imaging alone as many tumors such as giant cell tumors, aneurysmal bone cysts, FD, non-ossifying fibroma, chondromyxoid fibroma, and eosinophilic granuloma have similar radiological appearances [ 4 , 11 ].…”

“…Bone biopsy remains the gold standard for DF diagnosis [ 2 , 4 ]. The histopathologic assessment is characterized by slender fibroblastic to reticular cells without pleomorphism or mitotic activity embedded in a copious collagenous matrix [ 14 ].…”

“…Desmoplastic fibroma (DF) is an extremely rare primary bone tumor characterized by a nonmetastatic aggressive behavior [ 1 ]. These locally destructive neoplasms represent around 0.06% of all bone tumors and 0.03% of benign bone tumors [ 1 , 2 ]. DF was first described in 1958 by Jaffe, who highlighted their histological resemblance to a desmoid tumor [ 3 ].…”

Unraveling the challenges in the diagnosis and management of desmoplastic fibroma of the mandible–a case report

“…Jaffe originally described an aggressive fibromatosis of the femur, tibia, and scapula that was largely osseous in origin in 1958 [ 4 ]. The incidence of this tumor is less than 1% compared to other tumors of the bone [ 5 , 6 ]. Desmoplastic Fibroma has the potential to impact any bone in the body, it is frequently observed in the mandible (22%), followed by the femur (15%), pelvic bones (13%), radius (12%), and tibia (9%) [ 3 , 7 ].…”