Desmoplastic Fibroma of the Rib

Desmoplastic fibroma is a rare primary tumor of bone that histologically and biologically mimics the extra‐abdominal desmoid tumor of soft tissue. This study reviews 27 cases of desmoplastic fibroma, consisting of 9 from the Mayo Clinic files and 18 from our consultation files. There was a male predominance, and 74% of the patients were in the first 3 decades of life. The most frequent sites of involvement were the metaphysis of long bones and the mandible. Radiographically, the tumors were lucent, expansile lesions with well‐defined margins. Histologically, they contained slender spindle cells and various amounts of collagen fibers. En bloc resection is the treatment of choice because a high incidence of recurrence was noticed after lesional curettage.

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Desmoplastic fibroma of the rib with cystic change: a case report and literature review

Okubo

Saito

Takagi

et al. 2013

Skeletal Radiol

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Desmoplastic fibroma (DF) is a rare, locally aggressive, solitary tumor microscopically composed of well-differentiated myofibroblasts with abundant dense collagen deposition. The most common sites are the long tubular bones and mandible. To our knowledge, only five cases of DF in the ribs have been reported. Here, we report a case of DF in this rare location with unusual radiological findings. A 40-year-old man presented with a 4-year history of swelling of the right chest wall. Radiographs revealed a mass at the right 9th rib, and computed tomography demonstrated a mass of 14 × 12 × 8 cm at the right 9th rib with expanded cystic change and marked calcification that appeared to have arisen from the bone. Open biopsy suggested DF. Total excision was performed, and the chest wall was reconstructed. The surgical specimen was a yellowish tumor with multilocular cystic change containing a viscous liquid. The tumor was composed of a proliferation of less-atypical spindle-shaped cells in a collagenous background. The cystic change was observed in the extra-osseous lesion. No β-catenin cytoplasmic/nuclear accumulation was detected, and no β-catenin or GNAS genetic mutations were detected. A final diagnosis of DF was made on the basis of the pathological and radiological findings. The patient was successfully treated with total excision of the tumor with no evidence of recurrence 6 months after surgery.

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Desmoplastic Fibroma of the Rib

Abstract: We present a 17-year-old male patient who had a desmoplastic fibroma. The diagnosis is histologically verified and differentiated from other tumors. The rare localization of this benign lesion in the rib is of particular interest.

Cited by 6 publications

References 3 publications

Desmoplastic fibroma of the bone: A report of two patients, review of the literature, and therapeutic implications

Desmoplastic fibroma of the bone: A report of two patients, review of the literature, and therapeutic implications

Desmoplastic fibroma of bone

Desmoplastic fibroma of the rib with cystic change: a case report and literature review

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