Angiomatoid fibrous histiocytoma

Angiomatoid fibrous histiocytoma (AFH) is a rare type of soft tissue sarcoma that primarily affects children and young adults. It was first described in 1979 by Enzinger.

Epidemiology

AFH accounts for approximately 0.3% of all soft tissue tumors. It is most commonly diagnosed in children and young adults, with a median age at diagnosis of 10 years. However, it can occur at any age.

Pathology

AFH is characterized by the presence of blood-filled spaces, which are surrounded by fibrous tissue. The tumor cells are typically spindle-shaped and have a histiocytic appearance. The tumor is often encapsulated and may be associated with a fibrous pseudocapsule.

Clinical Presentation

Patients with AFH typically present with a painless, slowly growing mass. The most common locations for the tumor are the extremities, followed by the head and neck region. However, it can occur in any part of the body.

Diagnosis

The diagnosis of AFH is based on the clinical presentation, imaging studies, and histopathological examination. Immunohistochemistry is often used to confirm the diagnosis. The tumor cells are typically positive for desmin and CD68, and negative for S100 protein and CD34.

Treatment

The mainstay of treatment for AFH is surgical resection. Chemotherapy and radiotherapy may be used in cases where complete surgical resection is not possible or in cases of recurrent disease.

Prognosis

The prognosis of AFH is generally good, with a 5-year survival rate of over 90%. However, local recurrence and distant metastasis can occur, particularly in cases with incomplete surgical resection.

See Also

• Soft tissue sarcoma
• Histiocytoma
• Desmin
• CD68