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Atypical fibroxanthoma

Authors
Fiona Zwald, MD, MRCPI
Ian A Maher, MD
Section Editor
June K Robinson, MD
Deputy Editor
Rosamaria Corona, MD, DSc

INTRODUCTION

Atypical fibroxanthoma (AFX) is an uncommon, pleomorphic, spindle cell cutaneous malignancy that most commonly presents as a solitary red or pink papule or nodule on the head or neck (picture 1A) [1]. Exposure to ultraviolet light most likely contributes to the development of AFX.

The relationship between AFX and undifferentiated pleomorphic sarcoma (UPS, a soft tissue neoplasm that shares many histologic features with AFX) is unclear. While some authors consider AFX a less aggressive, superficial variant of undifferentiated pleomorphic sarcoma (UPS), many others view AFX as a distinct malignancy.

The recommended treatment for AFX is surgical removal of the entire tumor with Mohs surgery or wide local excision. AFX generally has a good prognosis. Metastasis is a rare event.

The clinical features, diagnosis, and management of AFX are discussed here. Undifferentiated pleomorphic sarcoma is reviewed separately. (See "Clinical presentation, histopathology, diagnostic evaluation, and staging of soft tissue sarcoma".)

EPIDEMIOLOGY

Although it is accepted that atypical fibroxanthoma (AFX) is an uncommon tumor, the incidence of AFX is unknown. The results of a retrospective study of surgical logs for Mohs surgery (a common treatment for AFX) from five practices in Texas offers support for the infrequent occurrence of this tumor. Out of 42,279 skin cancers treated with Mohs surgery, only 105 (0.2 percent) were AFX [2].

                      

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Literature review current through: Nov 2016. | This topic last updated: Wed Nov 09 00:00:00 GMT 2016.
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