Atypical Fibroxanthoma 

  • Author: Forrest C Brown, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Jun 2, 2010
 

Background

Atypical fibroxanthoma (AFX) is a tumor that occurs primarily in older individuals after the skin of the head and neck has been damaged significantly by sun exposure and/or therapeutic radiation. Clinically, lesions usually are suggestive of malignancy because they arise rapidly (over just a few weeks or months) in skin in which other skin cancers have been found and treated. When this clinical impression is combined with highly anaplastic pathology, misdiagnosis can result in unnecessary and extensive surgery and radiation. The image below demonstrates the typical clinical presentation of an atypical fibroxanthoma.

Red, beefy, sessile nodule typical of clinical preRed, beefy, sessile nodule typical of clinical presentation of atypical fibroxanthoma. Note the markedly sun-damaged skin with solar telangiectasias. Courtesy of Capt James Steger, MC, USN, US Naval Hospital, San Diego.

Histologically, lesions show a highly atypical and pleomorphic cellular appearance, but they typically respond to simple excision. Clinicopathologic correlation is essential. Factors important to consider are lesion location, patient age, histopathologic appearance, and the observation that the tumor arises from the dermis, not the fat. Many AFX tumors may represent a superficial form of malignant fibrous histiocytoma (MFH) with a much better prognosis. Some cases may represent primary squamous cell carcinoma (SCC) that fails to express keratin.

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Epidemiology

Sex

Male-to-female ratio is equal.

Age

In one study, age ranged from 13-95 years with a mean age of 69 years.

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Contributor Information and Disclosures
Author

Forrest C Brown, MD  Clinical Professor of Dermatology, University of Texas Southwestern Medical School; Section Chief, Department of Dermatology, Medical City Dallas Hospital

Disclosure: Nothing to disclose.

Specialty Editor Board

Carrie L Kovarik, MD  Assistant Professor of Dermatology, Dermatopathology, and Infectious Diseases, University of Pennsylvania School of Medicine

Carrie L Kovarik, MD is a member of the following medical societies: Alpha Omega Alpha

Disclosure: Nothing to disclose.

Richard P Vinson, MD  Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association

Disclosure: Nothing to disclose.

Rosalie Elenitsas, MD  Herman Beerman Associate Professor of Dermatology, University of Pennsylvania School of Medicine; Director, Penn Cutaneous Pathology Services, Department of Dermatology, University of Pennsylvania Health System

Rosalie Elenitsas, MD is a member of the following medical societies: American Academy of Dermatology and American Society of Dermatopathology

Disclosure: Lippincott Williams Wilkins Royalty Textbook editor; DLA Piper Consulting fee Consulting

Joel M Gelfand, MD, MSCE  Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania

Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology

Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds Investigator; Genentech Grant/research funds investigator; Centocor Consulting fee Consulting; Abbott Grant/research funds investigator; Abbott Consulting fee Consulting; Novartis investigator; Pfizer Grant/research funds investigator; Celgene Consulting fee DMC Chair; NIAMS and NHLBI Grant/research funds investigator

Chief Editor

Dirk M Elston, MD  Director, Department of Dermatology, Geisinger Medical Center

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

References

  1. Zalla MJ, Randle HW, Brodland DG, et al. Mohs surgery vs wide excision for atypical fibroxanthoma: follow-up. Dermatol Surg. Dec 1997;23(12):1223-4. [Medline].

  2. Ang GC, Roenigk RK, Otley CC, Kim Phillips P, Weaver AL. More than 2 decades of treating atypical fibroxanthoma at mayo clinic: what have we learned from 91 patients?. Dermatol Surg. May 2009;35(5):765-72. [Medline].

  3. Calonje E, Wadden C, Wilson-Jones E, Fletcher CD. Spindle-cell non-pleomorphic atypical fibroxanthoma: analysis of a series and delineation of a distinctive variant. Histopathology. Mar 1993;22(3):247-54. [Medline].

  4. Champion R, Burton JL, Burns DA, Breathnach SM, eds. Rook/Wilkinson/Ebling Textbook of Dermatology. Vol 3. 6th ed. Oxford: Blackwell Science;1998:2352-3.

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  7. Giuffrida TJ, Kligora CJ, Goldstein GD. Localized cutaneous metastases from an atypical fibroxanthoma. Dermatol Surg. Dec 2004;30(12 Pt 2):1561-4. [Medline].

  8. Grosso M, Lentini M, Carrozza G, Catalano A. Metastatic atypical fibroxanthoma of skin. Pathol Res Pract. Jun 1987;182(3):443-7. [Medline].

  9. Hafner J, Kunzi W, Weinreich T. Malignant fibrous histiocytoma and atypical fibroxanthoma in renal transplant recipients. Dermatology. 1999;198(1):29-32. [Medline].

  10. Lazova R, Moynes R, May D, Scott G. LN-2 (CD74). A marker to distinguish atypical fibroxanthoma from malignant fibrous histiocytoma. Cancer. Jun 1 1997;79(11):2115-24. [Medline].

  11. Lee CS, Chou ST. p53 protein immunoreactivity in fibrohistiocytic tumors of the skin. Pathology. Aug 1998;30(3):272-5. [Medline].

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  15. Requena L, Sangueza OP, Sanchez Yus E, Furio V. Clear-cell atypical fibroxanthoma: an uncommon histopathologic variant of atypical fibroxanthoma. J Cutan Pathol. Mar 1997;24(3):176-82. [Medline].

  16. Rudisaile SN, Hurt MA, Santa Cruz DJ. Granular cell atypical fibroxanthoma. J Cutan Pathol. Apr 2005;32(4):314-7. [Medline].

  17. Silvis NG, Swanson PE, Manivel JC, et al. Spindle-cell and pleomorphic neoplasms of the skin. A clinicopathologic and immunohistochemical study of 30 cases, with emphasis on "atypical fibroxanthomas". Am J Dermatopathol. Feb 1988;10(1):9-19. [Medline].

  18. Starink TH, Hausman R, Van Delden L, Neering H. Atypical fibroxanthoma of the skin. Presentation of 5 cases and a review of the literature. Br J Dermatol. Aug 1977;97(2):167-77. [Medline].

  19. Wilson PR, Strutton GM, Stewart MR. Atypical fibroxanthoma: two unusual variants. J Cutan Pathol. Apr 1989;16(2):93-8. [Medline].

  20. Worrell JT, Ansari MQ, Ansari SJ, Cockerell CJ. Atypical fibroxanthoma: DNA ploidy analysis of 14 cases with possible histogenetic implications. J Cutan Pathol. Jun 1993;20(3):211-5. [Medline].

  21. Bugatti L, Filosa G. Dermatoscopic features of cutaneous atypical fibroxanthoma: three cases. Clin Exp Dermatol. Dec 2009;34(8):e898-900. [Medline].

 
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Red, beefy, sessile nodule typical of clinical presentation of atypical fibroxanthoma. Note the markedly sun-damaged skin with solar telangiectasias. Courtesy of Capt James Steger, MC, USN, US Naval Hospital, San Diego.
Microscopic view of atypical fibroxanthoma. Note the large abnormal-appearing cells in a field of spindle cells. Courtesy of Capt James Steger, MC, USN, US Naval Hospital, San Diego.
Table. Antibody Panels in Tumors
AntibodyAFXMFHSCCDFSPSpindle



MM*



Leiomyosarcoma
VimentinRRNRRR
CytokeratinNNRNNN
S-100NNNNRN
Desmin or smooth muscle actinNNNNNR
*Spindle cell malignant melanoma
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