Desmoplastic fibroma

Desmoplastic fibroma
Synonyms
Pronounce	N/A
Specialty	N/A
Symptoms	Pain, swelling, pathological fracture
Complications
Onset
Duration
Types
Causes	Unknown
Risks
Diagnosis	X-ray, MRI, CT scan, biopsy
Differential diagnosis	Fibrous dysplasia, non-ossifying fibroma, osteosarcoma
Prevention
Treatment	Surgical resection
Medication
Prognosis	Generally good with complete resection
Frequency	Rare
Deaths

Desmoplastic fibroma is a rare, benign, locally aggressive fibrous neoplasm that typically arises in the metaphysis of long bones or in the mandible. It is a type of fibroma that is characterized by the presence of abundant collagen and a relatively small number of fibroblasts.

Clinical Presentation

Patients with desmoplastic fibroma often present with pain and swelling in the affected area. The tumor may also cause pathological fractures. The age of onset is usually in the first two decades of life, but it can occur at any age.

Diagnosis

The diagnosis of desmoplastic fibroma is based on the clinical presentation, radiographic findings, and histopathological examination. Radiographically, the tumor appears as a lytic, well-defined, non-calcified mass. Histologically, it is composed of spindle-shaped fibroblasts embedded in a dense collagenous stroma.

Treatment

The treatment of choice for desmoplastic fibroma is wide surgical excision. However, due to the aggressive nature of the tumor and its tendency to recur, complete removal may not always be possible. In such cases, adjuvant therapies such as radiation therapy or chemotherapy may be considered.

Prognosis

The prognosis for patients with desmoplastic fibroma is generally good, as the tumor is benign and does not metastasize. However, the local aggressiveness of the tumor and the potential for recurrence can lead to significant morbidity.

See Also

• Fibroma
• Bone tumor
• Radiation therapy
• Chemotherapy

References

• Fletcher, CDM, Bridge JA, Hogendoorn P, Mertens F. WHO Classification of Tumours of Soft Tissue and Bone. IARC Press: Lyon 2013.