Cases reported "Fibroma, Desmoplastic"

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1/17. Desmoplastic fibroma of the mandible: report of a case.

    This paper describes a case of desmoplastic fibroma in a 4-year-old patient with a history of a small slowly growing swelling at the right angle of the mandible over a 3-month period. Desmoplastic fibroma was diagnosed on histological and immunohistochemical bases. The lesion responded well to thorough curettage and has not shown signs of recurrence 3 years after the surgical intervention. The clinical picture, the pathology and the management of the case are described, and the differential diagnosis and treatment are discussed.
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2/17. MR findings of desmoplastic fibroma of the spine. A case report.

    We report on the MR imaging findings in a case of spinal desmoplastic fibroma (DF). DF of the bone is a rare, locally aggressive tumor. It is commonly located in long bones, pelvis or mandible. DF involving the spine is extremely rare and difficult to distinguish from other bony lesions such as giant cell tumor, chordoma and fibrous dysplasia of the spine. This case of DF of the spine showed MR findings similar to those of DF arising in the metaphysis of a long bone.
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3/17. Desmoplastic fibroma of the temporal bone.

    An unusual case of a desmoplastic fibroma of the temporal bone is presented. Although classified benign, this intraosseous lesion exhibits local aggressiveness and has a high potential for recurrence. This rare condition occurs predominantly in the mandible and in the long bones and is seldom seen in the calvarium. Here we add another case to the previously described eight in the skull and this is the fourth such case reported in the temporal bone. The clinical features, radiology, histopathology and the therapeutic considerations of this lesion in a 72-year-old female are discussed. In addition, a literature review of all the cases affecting the skull bones is presented.
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4/17. Desmoplastic fibroma of the rib.

    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.
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5/17. Desmoplastic fibroma of the cranium: case report and review of the literature.

    OBJECTIVE AND IMPORTANCE: Desmoplastic fibroma is a benign bone tumor that can be locally aggressive. It usually occurs in the long bones and mandible. We report on a patient with a desmoplastic fibroma arising in the temporal bone and review previously published cases of desmoplastic fibroma originating within the cranium. CLINICAL PRESENTATION: A 43-year-old woman presented with a 12-year history of progressively worsening head asymmetry. magnetic resonance imaging and computed tomography demonstrated a mass originating from the bone and involving the adjacent soft tissues. INTERVENTION: A temporal parietal craniectomy was performed with excision of a large tumor involving the bone. An acrylic cranioplasty was used to replace the bone. Pathological examination of the lesion identified desmoplastic fibroma of the cranium. After surgery, the patient's cranial asymmetry was corrected. CONCLUSION: Desmoplastic fibroma of the cranium is rare. Surgical resection is the treatment of choice.
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6/17. Desmoplastic fibroma of bone: case report.

    Desmoplastic fibroma of bone is an exceedingly rare tumor that was first described by Jaffe in 1958 [1]. It has a high incidence of local recurrence after surgical resection. It is usually seen in young patients and involves mandible and long bones. It has histological resemblance to the desmoid tumor of the abdominal wall. We report a surgically proven case of desmoplastic fibroma of bone with local aggressiveness and recurrence during follow-up. The radiograph, CT imaging features, radiological and pathological differential diagnosis of the case are described, and literature is briefly reviewed.
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7/17. Enhancement of the fibula free flap by alveolar distraction for dental implant restoration: report of a case.

    We describe the utilization of distraction osteogenesis in the free fibular microvascular bone graft to the mandible for increasing bone height for future osseointegrated dental implants. Successful reconstruction of a resected mandible requires restoration of both function and esthetic form. Although current reconstructive techniques restore anterior-posterior and lateral projection, often the graft's vertical height is not sufficient for the placement of osseointegrated dental implants and subsequent oral prosthesis. The patient was a sixteen-year-old male who was found to have a large desmoplastic fibroma of the left mandible, which was resected. The defect was successfully restored with a free fibular microvascular bone graft and reconstruction plate. Nevertheless, the patient had persistent problems with mastication and it was decided to perform a segmental osteotomy of the neomandible. Two internal vertical distraction devices were then placed in the mandible. The appliances were then activated five days postoperative, twice a day, for a total of 14 days. At that time 1.5 cm of distraction had occurred and the patient was placed in a consolidation phase for four months. The patient then had sufficient bone height and was restored with 8 osseointegrated dental implants.
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8/17. Three-level en bloc spondylectomy for desmoplastic fibroma of the thoracic spine: a case report.

    STUDY DESIGN: A case of desmoplastic fibroma of the thoracic spine treated by a three-level en bloc spondylectomy is reported. OBJECTIVES: To present a rare case of desmoplastic fibroma of the spine, and to emphasize the importance of at least a marginal resection of this tumor entity. SUMMARY OF BACKGROUND DATA: Desmoplastic fibroma is a rare tumor, with the literature reporting approximately 220 cases of the disorder in patients younger than 30 years of age. This disorder has a predilection for the mandible and the meta-diaphyses of long bones. A location of desmoplastic fibroma in the spine has been reported in only a few cases. Desmoplastic fibroma has a high tendency of local recurrence, especially after intralesional resection. methods: The reported case is that of a 14-year-old girl with a desmoplastic fibroma of the 9th, 10th, and 11th vertebrae. After confirmation of the diagnosis by CT-guided biopsy, a three-level en bloc spondylectomy with marginal resection of the desmoplastic fibroma was performed from the posterior approach. Stabilization was achieved with a multilevel pedicle screw instrumentation, and an autologous fibula was used for reconstruction. RESULTS: At this writing, 31 months after surgery, the girl has no evidence of recurrence and is pain free. CONCLUSIONS: Wide resection of tumors located in the spine actually is impossible to achieve because of the anatomic relations to the spinal cord, the major vessels, and the lung. As in the current case, a marginal resection is the maximal one to be achieved. Three of seven cases (43%) of desmoplastic fibroma in the spine treated by intralesional resection resulted in a local recurrence. These data clarify the importance of at least marginal resection of desmoplastic fibroma, if this is anatomically and technically possible. A local recurrence of desmoplastic fibroma in the spine can be impossible to treat surgically with a curative intention without a significant loss of function.
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9/17. Desmoplastic fibroma-like tumor of maxillofacial region associated with tuberous sclerosis.

    Desmoplastic fibroma is a rare primary tumor of bone that histologically and biologically mimics the extra-abdominal desmoid tumor of soft tissue. It usually presents in patients during the first three decades of life and often involves the mandible or long bones of the skeleton. Its clinical behavior is characterized by a locally aggressive, infiltrating, and destructing course, often with invasion of surrounding tissues but without metastasis. We present herein the clinicopathological features of a desmoplastic fibroma-like tumor involving the left maxillofacial region in a 14-year-old Hispanic boy with tuberous sclerosis.
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10/17. Desmoplastic fibroma of the mandible in an infant.

    We report the CT and MR imaging findings of a case of a desmoplastic fibroma of the mandible in a 6-month-old girl who presented with a rapidly enlarging facial mass and who represents one of the youngest reported children with this rare tumor. Although imaging is non-specific and these lesions are best distinguished histologically, the desmoplastic fibroma should be considered when there is an occurrence of any bubbly or cystic expansile mandibular lesion presenting in childhood.
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