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Atypical fibroxanthoma
See also in: Hair and Scalp
Other Resources UpToDate PubMed

Atypical fibroxanthoma

See also in: Hair and Scalp
Contributors: Gabriela Cobos MD, Belinda Tan MD, PhD, Susan Burgin MD
Other Resources UpToDate PubMed


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.


C49.9 – Malignant neoplasm of connective and soft tissue, unspecified

254754005 – Atypical fibroxanthoma of skin

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Differential Diagnosis & Pitfalls

  • Dermatofibrosarcoma protuberans
  • Amelanotic melanoma
  • Cutaneous squamous cell carcinoma
  • Merkel cell carcinoma
  • Nodular basal cell carcinoma
  • Lobular capillary hemangioma (pyogenic granuloma)
  • Metastatic cutaneous carcinoma
  • Basosquamous carcinoma of skin
  • Undifferentiated pleomorphic sarcoma
  • Pleomorphic dermal sarcoma (PDS) – Intermediate in biologic behavior between AFX and undifferentiated pleomorphic sarcoma, PDS involves the subcutaneous tissue, may extend to muscle or fascia, and shows perineural or vascular invasion.

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Last Reviewed:07/20/2020
Last Updated:07/28/2020
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Atypical fibroxanthoma
See also in: Hair and Scalp
A medical illustration showing key findings of Atypical fibroxanthoma : Face, Neck, Scalp
Clinical image of Atypical fibroxanthoma - imageId=4211299. Click to open in gallery.  caption: 'An ulcerated erythematous plaque with overlying hemorrhagic crusting at the temple.'
An ulcerated erythematous plaque with overlying hemorrhagic crusting at the temple.
Copyright © 2024 VisualDx®. All rights reserved.