Atypical fibroxanthoma (AFX) is a rare, low-grade sarcoma that most often occurs on sun-exposed skin of the head and neck in individuals older than 60 years. It presents as a solitary, red or pink, firm papule or nodule that grows rapidly over the course of several months. It may be ulcerated on presentation.

AFX may occur in individuals of any race, but most cases have been reported in individuals of Northern European descent. There is a male sex predilection.

Ultraviolet (UV) light exposure is considered a major risk factor for the development of AFX. Other proposed risk factors are radiation exposure, immunosuppression, burns, and trauma. Of note, the incidence of AFX in transplant patients is as high as 78 per 100 000 patients.

AFX carries a good prognosis overall. Recurrences typically occur within the first 3 years. Based on a review of 1500 published cases, the overall recurrence rate was 7.6%. Metastasis is rare, but when it occurs, the lymph nodes are often the first site of involvement. In this same review, the disease-specific survival was 98% at 5, 10, and 20 years.

Pediatric Patient Considerations:
A few cases of AFX have been reported in children with xeroderma pigmentosum.