Atypical Fibroxanthoma

ByVinod E. Nambudiri, MD, MBA, EdM, Harvard Medical School
Reviewed ByJoseph F. Merola, MD, MMSc, UT Southwestern Medical Center
Reviewed/Revised Modified Mar 2026
v21366914
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Atypical fibroxanthoma is a low-grade sarcoma of the skin.

(See also Overview of Skin Cancer.)

Atypical fibroxanthomas are superficial, low-grade dermal sarcomas that typically present as rapidly growing, red or pink nodules on chronically sun-exposed skin. They are rare among nonmelanoma skin cancers. They most commonly occur on the head and neck of older patients and have a male predilection. They appear similar to other nonmelanoma skin cancers, as nonhealing or tender pink-red skin papules or nodules.

The diagnosis of atypical fibroxanthoma is with biopsy.

Tumors are excised, or Mohs micrographic surgery—in which tissue borders are progressively excised until specimens are tumor-free as determined by microscopic examination during surgery—is performed if clinically appropriate. Recurrence occurs in approximately 5% of patients (1). Metastasis and disease-associated mortality is highly unlikely.

General reference

  1. 1. von Dannecker R, de Oliveira LML, Lapenda I, et al. Clinical Outcomes and Prognosis in Atypical Fibroxanthoma: A Systematic Review and Meta-Analysis. Int J Dermatol. 2025 Sep;64(9):1592-1598. doi: 10.1111/ijd.17841. Epub 2025 May 11. PMID: 40350546.

Prevention of Atypical Fibroxanthoma

Because atypical fibroxanthomas are associated with ultraviolet (UV) radiation exposure, a number of measures are recommended to limit exposure (eg, sun avoidance measures, use of protective clothing, use of sunscreen). For more detailed information, see Prevention of Effects of Sun Exposure.

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