Diagnosis Of Desmoplastic Fibroma Research Articles

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

Desmoplastic fibroma (DF) is an uncommon bone tumor that originates from the mesenchymal tissue and despite being benign, exhibits aggressive behavior locally. The following report describes the case of a 7-year-old boy with a rapidly enlarging swelling on the right side of the mandible. After a thorough clinical examination, radiographic imaging, and histopathological analysis, the diagnosis of DF was confirmed. Treatment planning was formulated considering both the tumor’s tendency for local recurrence and the patient’s well-being. Due to the patient’s young age, segmental resection was not deemed appropriate, and an aggressive curettage and enucleation of the lesion followed by the bone graft was performed instead. The patient was kept under close follow-up for the first month of post-surgery and later reviewed after 3, 6, 9, and 12 months, respectively. Good bone healing was observed on radiographs. The patient did not show any signs of recurrence based on clinical or radiographic assessments and did not exhibit any neurosensory deficits as well.

Desmoplastic Fibroma: a benign neoplasm mimicking a malignancy

A four-year-old African male patient presented to our clinic with a two-month history of a fi rm, warm lesion involving the right side of his face. The lesion presented as a bleeding papillary mass in the 85/46 region that extended to the floor of the mouth, causing displacement of the tongue (Figure 1A-B). Upon cone-beam computed tomography (CBCT) examination, an expansile homogenous intermediate density lesion was noted with complete destruction of the bucco-lingual cortical bone. Discernible extensions were identified in the anteroposterior plane from the region of the 84 to the posterior mandible involving the right corpus, developing 46, angle and ramus but spared the condyle and coronoid processes. The lesion extended inferiorly below the epiglottis and showed a reduction of the hypopharynx. Marked bucco-lingual expansion with soft tissue swelling was noted (Figure 2A-D). An incisional biopsy was performed under general anaesthesia, and histological examination rendered a diagnosis of desmoplastic fi broma (DF).

DESMOPLASTIC FIBROMA OF MAXILLA: A CASE REPORT

Desmoplastic fibroma (DF) is an extremely rare aggressive benign bone tumor, and it represents 0.1% of all primary bone tumors. We reported a case of a 53-year-old feoderm man. In the extraoral examination, a discrete volume increase was observed in the genic region that was painless under pressure; magnetic resonance imaging and cone beam computed tomography showed an expansive left maxillary sinus lesion with involvement of the homolateral zygomatic bone. The patient was submitted to previous incisional biopsy with histopathologic diagnosis of lipoma being referred for surgical removal. In the intraoperative period the noncompatibility with the aforementioned diagnosis was verified; thus, a new histopathologic examination of the specimens was requested that resulted in the diagnosis of DF. The subsequent control is performed annually with the help of cone beam computed tomography. Currently, the patient is healthy and asymptomatic.

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

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 × 8cm 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 6months after surgery.

Desmoplastic Fibroma of the Cervical Spine

A case report and literature review are presented. To present a rare case of desmoplastic fibroma (DF) in the cervical spine and discuss the diagnosis and treatment of this disorder. DF is an extremely rare primary tumor of bone, especially in cervical spine. It is a benign but locally aggressive tumor of the connective tissue. It has a predilection for the mandible and the metadiaphyses of long bones. It has a propensity for local recurrence, especially after intralesional resection. We report on a 70-year-old woman with a DF of the fourth and fifth cervical vertebrae. The patient complained of progressive neck pain and limitation of activity. After radiologic evaluation, a mass lesion was found on the fourth and fifth cervical vertebral bodies. A 2-level corpectomy with wide marginal resection of the tumor was performed through the anterior approach, and the histopathologic examination yielded the diagnosis of DF. Reconstruction was achieved with a long titanium mesh and locking plate with autologous iliac crest bone graft. After surgery, pain was completely relieved, and neurologic function was normal. The postoperative course was uneventful, and the patient has been well without recurrence for 35 months of follow-up evaluation. Patients with DF of the cervical spine may present with the arm and neck pain mimicking cervical disc disease. High index of suspicion by the clinicians must be practiced to make the appropriate diagnosis, and histologic confirmation of the diagnosis is essential. Surgical resection with wide margins is the preferred treatment.

Le fibrome desmoplastique ou fibrome desmoïde osseux: À propos de 2 nouveaux cas d’une tumeur osseuse rare

Desmoplastic fibroma or desmoid bone tumor is a rare tumor described for the first time by Jaffe in 1958. It accounts for 0.1 to 0.3% of all benign bone tumors. To date, about 150 cases involving the locomotor system have been reported. Histology is required for certain diagnosis of desmoplastic fibroma. The tumor is composed of sparse fibroblasts in a rich background of collagen fibers, a histological presentation exactly the same as soft tissue desmoid fibroma. We report two new cases of desmoplastic fibroma of the locomotor system. The observations illustrate the radiological diagnosis. MRI was used to search for local extension in bone or soft tissues. Biopsy is necessary to confirm the diagnosis. The histological presentation may be difficult to recognized and distinguish from low grade fibrosarcoma. After treatment, the rate of local recurrence is high in the event of partial resection. Surgery is the optimal treatment, with tumor resection as wide as possible depending on the localization.

Desmoplastic fibroma of bone

A 5-year-old Chinese boy had dull pain in his right knee for about one year. An intraosseous osteolytic lesion in the metaphysis of the right lower femur was curetted. The diagnosis of desmoplastic fibroma, possibly the first in a Hong Kong Chinese, was based on the clinical, radiological and histological findings. About one year later the only abnormality was a small rounded radiological lucency, static for 4 months. The fibroblastic nature of the lesion and its tendency to local recurrence support a fibromatosis of intraosseous origin.