ContentsSynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyReferencesView all Images (22)
Atypical fibroxanthoma
See also in: Hair and Scalp
Print
Other Resources UpToDate PubMed

Atypical fibroxanthoma

See also in: Hair and Scalp
Print Images (22)
Contributors: Gabriela Cobos MD, Belinda Tan MD, PhD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Atypical fibroxanthoma (AFX) is a rare, low-grade sarcoma that most often occurs on sun-exposed skin of the head and neck in individuals above the age of 60. 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.

Codes

ICD10CM:
C44.300 – Unspecified malignant neoplasm of skin of unspecified part of face

SNOMEDCT:
254754005 – Atypical fibroxanthoma of skin

Look For

Subscription Required

Diagnostic Pearls

Subscription Required

Differential Diagnosis & Pitfalls

Best Tests

Subscription Required

Management Pearls

Subscription Required

Therapy

Subscription Required

References

Subscription Required

Last Reviewed: 06/12/2017
Last Updated: 10/01/2019
Copyright © 2019 VisualDx®. All rights reserved.
Atypical fibroxanthoma
See also in: Hair and Scalp
Print 22 Images Filter Images
View all Images (22)
(with subscription)
 Reset
Atypical fibroxanthoma : Cheek, Face, Plaque with ulcer, Smooth papule, Vertex scalp
Clinical image of Atypical fibroxanthoma
Copyright © 2019 VisualDx®. All rights reserved.