title: Desmoplastic fibroma of the rib with cystic change: a case report and literature review.
abstract: 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.
authors.affiliation: Department of Orthopaedic Surgery, School of Medicine, Juntendo University, Tokyo, Japan.
authors.forename: Taketo
authors.initials: T
authors.lastname: Okubo
authors.forename: Tsuyoshi
authors.initials: T
authors.lastname: Saito
authors.forename: Tatsuya
authors.initials: T
authors.lastname: Takagi
authors.forename: Yoshiyuki
authors.initials: Y
authors.lastname: Suehara
authors.forename: Kazuo
authors.initials: K
authors.lastname: Kaneko
mesh_terms: D000328:Adult; D001845:Bone Cysts / Q000175:diagnosis* / Q000209:etiology*; D001859:Bone Neoplasms / Q000150:complications* / Q000175:diagnosis*; D018220:Fibroma, Desmoplastic / Q000150:complications* / Q000175:diagnosis*; D006801:Humans; D008297:Male; D012272:Ribs / Q000000981:diagnostic imaging* / Q000473:pathology; D014057:Tomography, X-Ray Computed / Q000379:methods