Desmoplastic fibroblastoma

Desmoplastic Fibroblastoma
Synonyms	Collagenous fibroma
Pronounce	N/A
Specialty	N/A
Symptoms	Painless, slow-growing mass
Complications	Rarely any
Onset	Typically in adults
Duration	Chronic
Types	N/A
Causes	Unknown
Risks
Diagnosis	Histopathology
Differential diagnosis	Fibroma, Fibromatosis, Sarcoma
Prevention	N/A
Treatment	Surgical excision
Medication	N/A
Prognosis	Excellent
Frequency	Rare
Deaths	N/A

Overview

Desmoplastic fibroblastoma, also known as collagenous fibroma, is a rare, benign soft tissue tumor characterized by a proliferation of spindle-shaped fibroblasts within a collagenous stroma. It typically presents as a painless, slow-growing mass and is most commonly found in the subcutaneous tissue or skeletal muscle of adults.

Epidemiology

Desmoplastic fibroblastoma is a rare condition, with a slight male predominance. It most frequently occurs in middle-aged adults, although cases have been reported across a wide age range. The tumor can arise in various anatomical locations, but it is most commonly found in the upper extremities, shoulders, and neck.

Pathophysiology

The exact pathogenesis of desmoplastic fibroblastoma is not well understood. It is characterized by the proliferation of spindle-shaped fibroblasts and myofibroblasts embedded in a dense collagenous matrix. The tumor is well-circumscribed but not encapsulated, and it does not invade surrounding tissues.

Clinical Presentation

Patients with desmoplastic fibroblastoma typically present with a painless, slow-growing mass. The tumor is usually well-defined and firm to palpation. It is often discovered incidentally, as it rarely causes symptoms unless it compresses nearby structures.

Diagnosis

The diagnosis of desmoplastic fibroblastoma is primarily based on histopathological examination. Imaging studies such as MRI or CT scan may be used to assess the extent of the lesion, but they are not diagnostic. A biopsy is required to confirm the diagnosis.

Histopathology

Microscopically, desmoplastic fibroblastoma is characterized by a proliferation of spindle-shaped fibroblasts and myofibroblasts within a dense, collagenous stroma. The cells are typically bland, with no significant atypia or mitotic activity. Immunohistochemical staining is often used to differentiate it from other soft tissue tumors.

Differential Diagnosis

The differential diagnosis for desmoplastic fibroblastoma includes:

• Fibroma
• Fibromatosis
• Nodular fasciitis
• Sarcoma

Treatment

The treatment of choice for desmoplastic fibroblastoma is surgical excision. The tumor is well-circumscribed, which allows for complete removal with clear margins. Recurrence is rare following adequate surgical resection.

Prognosis

The prognosis for patients with desmoplastic fibroblastoma is excellent. The tumor is benign and does not metastasize. Recurrence is uncommon after complete surgical excision.

See Also

• Fibroma
• Soft tissue tumor
• Benign tumor

External Links

• [Link to relevant medical resources]