eMedicine Specialties > Dermatology > Benign Neoplasms

Atypical Fibroxanthoma

Author: Forrest C Brown, MD, Clinical Professor of Dermatology, University of Texas Southwestern Medical School; Section Chief, Department of Dermatology, Medical City Dallas Hospital
Contributor Information and Disclosures

Updated: Aug 7, 2009

Introduction

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 pr...

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.

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Red, beefy, sessile nodule typical of clinical pr...

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.


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.

Sex

Male-to-female ratio is equal.

Age

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

Clinical

History

  • Typically, the patient presenting with atypical fibroxanthoma (AFX) is an older individual (mean age 69 y) with sun-damaged or radiation-damaged skin of the head, neck, and scalp.

Physical

  • Nodules are red, juicy, and dome shaped and they may be ulcerated, as in the image below. Lesions are usually located on skin that is red, thin, and telangiectatic, indicating previous significant sun or radiation damage. Some nodules are dark enough, due to deposits of hemosiderin, to be confused with a nodular melanoma.

  • Red, beefy, sessile nodule typical of clinical pr...

    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.

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    Red, beefy, sessile nodule typical of clinical pr...

    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.

  • Nodules primarily are located on the head and neck and in sun-exposed areas. In addition, lesions have been reported to occur on the trunk, extremities, and in sun-protected areas. The ratio of lesions that occur on the head and neck to lesions that occur in other areas is approximately 4:1.
  • Tumor size increases proportionately with duration of existence but rarely exceeds 3 cm in diameter.
  • Lesion growth typically is rapid, and patients usually seek medical advice within 6 months of onset.
  • In adult cases, skin underlying developing AFX lesions may be considered locally immunosuppressed. Recent reports showed an increased incidence of AFX in patients with AIDS and in patients who are immunosuppressed because of organ transplantation.
  • One case of localized cutaneous metastases has been reported after excision of the primary lesion. This seems to be extremely rare.

Causes

Sun exposure and/or therapeutic radiation that have caused significant skin damage are associated with the development of AFX. The tumor primarily occurs on the head or neck of older individuals.

More on Atypical Fibroxanthoma

Overview: Atypical Fibroxanthoma
Differential Diagnoses & Workup: Atypical Fibroxanthoma
Treatment & Medication: Atypical Fibroxanthoma
Follow-up: Atypical Fibroxanthoma
Multimedia: Atypical Fibroxanthoma
References
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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.

  5. Diaz-Cascajo C, Borghi S, Bonczkowitz M. Pigmented atypical fibroxanthoma. Histopathology. Dec 1998;33(6):537-41. [Medline].

  6. Fish FS. Soft tissue sarcomas in dermatology. Dermatol Surg. Mar 1996;22(3):268-73. [Medline].

  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].

  12. Leong AS, Milios J. Atypical fibroxanthoma of the skin: a clinicopathological and immunohistochemical study and a discussion of its histogenesis. Histopathology. May 1987;11(5):463-75. [Medline].

  13. Ma CK, Zarbo RJ, Gown AM. Immunohistochemical characterization of atypical fibroxanthoma and dermatofibrosarcoma protuberans. Am J Clin Pathol. Apr 1992;97(4):478-83. [Medline].

  14. Michie BA, Reid RP, Fallowfield ME. Aneuploidy in atypical fibroxanthoma: DNA content quantification of 10 cases by image analysis. J Cutan Pathol. Oct 1994;21(5):404-7. [Medline].

  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].

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Further Reading

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Keywords

atypical fibroxanthoma, AFX, sun exposure, photodamage, skin cancer, malignant fibrous histiocytoma, MFH, squamous cell carcinoma, squamous cell cancer, SCC, melanoma, nodular melanoma, sun damage, radiation exposure, radiation damage

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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.

Medical Editor

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.

Pharmacy Editor

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.

Managing Editor

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: Nothing to disclose.

CME Editor

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.

 
 
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