Case report 481: Benign intraosseous fibroma (desmoplastic fibroma) associated with Paget disease of the iliac bone.
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Desmoplastic fibroma is a relatively uncommon tumor and rarely involves the spine. The authors describe a 20-year-old woman with a thoracic epidural desmoplastic fibroma treated by complete resection and posterior spinal fusion. Four years after surgery, neither the tumor nor clinical symptoms have recurred. Thus, complete resection is considered necessary to treat this tumor.
Desmoplastic fibroma of bone is a rare benign tumor, first described by Jaffe in 1958. Since then approximately 80 cases have been reported in the long bones, although the tumor also involves the jaw bones, particularly the mandible. The plain film features consist of a lytic, expansile lesion, usually in the metaphysis of bone, sometimes extending into the soft tissues. We present two cases involving the long bones in which the correct diagnosis was suggested by computed tomography (CT). The CT appearance of the lesions is described.
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Desmoplastic fibroma of the long bones is very rare and has been mentioned in the literature, but there is no mention in the literature of desmoplastic fibroma occurring in relation to maxilla. The first case of desmoplastic fibroma of the maxilla is reported. Conservative surgery is recommended for desmoplastic fibroma of the maxilla to avoid facial deformity.
Intra-osseous fibromas of the jaw are classified by origin. Intra-osseous odontogenic fibromas have odontogenic epithelia, while desmoplastic fibromas do not. However, it is often difficult to determine the odontogenic origin for central fibromas. Three subjects with a diagnosis of intra-osseous fibroma were examined. Case 1 was a 35-year-old man found to have a panoramic radiograph from the right premolar to the mandibular ramus in the mandible that exhibited multilocular radiolucency. Within the radiolucency, small-radioopaque bodies were observed. Case 2 was a 13-year-old female, in whom a panoramic radiograph from the left premolar to the molar in the mandible showed multilocular radiolucency. Case 3 was a 51-year-old female who exhibited a heart-shaped radiolucency in the panoramic radiograph of the left first molar area in the mandible. We also reviewed the literature for previously reported cases of intra-osseous odontogenic and desmoplastic fibroma. In 64 cases of intra-osseous odontogenic fibroma and 68 cases of desmoplastic fibroma we extracted data on age, sex, location, and radiographic findings. Based on the analysis of the reported literature cases, re-evaluation of the patients in our study revealed that case 1 could be classified as desmoplastic fibroma, while cases 2 and 3 were intra-osseous odontogenic fibromas.
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.
Desmoplastic fibroma is a very rare primary tumor of bone, closely related to aggressive fibromatosis of soft tissue. Although considered a benign lesion, it is locally destructive, can extend into the soft tissues, and has a high rate of local recurrences after incomplete surgical excision. Recognition of this entity is important to ensure proper surgical treatment. According to the published data, the tumor is most common in the long tubular bones (56%), the mandible (26%), and the pelvis (14%). Rib involvement by desmoplastic fibroma is extremely rare, and to our knowledge, only 3 cases have been reported in the literature to date. We present the case of a desmoplastic fibroma in the rib of a 19-year-old man, adding a fourth case to the previously reported cases involving this unusual location. The clinical history and the radiological and pathologic findings are presented.
We observed clonal chromosome abnormalities in two fibrous soft tissue tumors diagnosed as collagenous fibroma (desmoplastic fibroblastoma). The involvement of the same band of the long arm of chromosome 11, 11q12, was observed in both tumors. The presence of hitherto unreported similar chromosomal abnormalities in this tumor supports the neoplastic nature of this lesion. In addition, a possible relationship with fibroma of tendon sheath, which also shows rearrangement of 11q12, is suggested. 11q12 might be a common genetic denominator of benign fibroblastic lesions, such as collagenous fibroma and fibroma of tendon sheath.
Desmoplastic fibroma is a rare, benign, fibroblastic tumour arising intra-osseously. A case in a 13 year-old male is described. A tumour, situated between the roots of the right mandibular canine and premolar, was excised in 1984. Morphologically, the characteristics of the tumour conformed well to those of a desmoplastic fibroma. The differential diagnosis of desmoplastic fibroma with reference to the histopathological and ultrastructural appearance of the tumour is discussed.
Collagenous fibroma (desmoplastic fibroblastoma) is a recently described tumor that may arise in the subcutaneous tissue or skeletal muscle. We report a case of collagenous fibroma, occurring on the forehead of a 67-year-old man. An awareness of this entity is necessary to avoid confusion with other soft tissue neoplasms, especially extraabdominal fibromatosis.
Collagenous fibroma (desmoplastic fibroblastoma) is a rare, benign tumor composed of spindle and stellate-shaped fibroblasts and myofibroblasts in a densely collagenous background. A t(2;11)(q31;q12) has been reported in one case of collagenous fibroma and a rearrangement of the 11q12 breakpoint in a second case. In the present study, we detected a t(2;11) identical to that previously described in a collagenous fibroma arising in the supraclavicular fossa of a 55-year-old man. This finding confirms the nonrandom association of t(2;11)(q31;q12) with collagenous fibroma.
Desmoplastic fibroma is an exceedingly rare primary bone tumor. Previous reports have not included the presentation of this tumor associated with the zygoma. This report describes the unusual presentation and surgical management of a desmoplastic fibroma of the zygoma.
BACKGROUND: Desmoplastic fibroma is a benign, locally aggressive, intraosseous neoplasm with a propensity for local recurrence. Desmoplastic fibroma most commonly originates within the mandible (70% of cases), and long bones with rare lesions reported in the maxillary, frontal, and parietal bones. We report two patients with desmoplastic fibroma arising within the temporal bone. STUDY DESIGN: Case report and literature review. PATIENTS: Two patients with desmoplastic fibroma with intracranial and extra cranial growth patterns are presented. Both were young females with aural fullness and decreased hearing. INTERVENTIONS: Diagnostic and therapeutic. RESULTS: Both tumors were surgically extirpated and the patients have remained disease free for 18-48 months. Neither tumor expressed estrogen or progesterone receptors. CONCLUSION: Desmoplastic fibroma is a highly invasive local destructive lesion which is best treated by nondestructive surgical intervention. Immunohistochemical analysis may demonstrate hormonal receptors, in which case Tamoxifen may reduce recurrence.
Desmoplastic fibromas of the facial skeleton are the bony counterparts to the soft-tissue desmoid tumors and are almost exclusively confined to the mandible. The diagnosis should be considered whenever a rapidly increasing swelling with little functional disability is noted in the mandible. Historical, clinical, radiologic, and histologic findings must be correlated to establish the correct diagnosis. Every effort must be made to distinguish these lesions from well-differentiated fibrosarcomas. An illustrative case is presented. The treatment of desmoplastic fibromas affecting the facial skeleton should be conservative. Curettage is recommended for small tumors. Wide resections with reconstruction should be reserved for larger lesions and for those that have recurred after conservative treatment.
Desmoplastic fibroma is an uncommon tumour of bone and has only exceptionally been reported in the jaws. This case report describes only the second example of a desmoplastic fibroma which involved the maxillary alveolus.
Desmoplastic fibroma is a rare, locally aggressive, benign tumor that is considered the skeletal counterpart of the desmoid tumor of soft tissues. Although the treatment of choice of desmoplastic fibroma is surgical excision, radiation therapy should be considered when surgery is not a viable option.
Desmoplastic fibroma is a benign but locally aggressive tumor arising usually from the mandible, pelvis and long bones with a potential for recurrence. We report a case of desmoplastic fibroma of the frontal bone in a young male.