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Overview

Practice Essentials

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 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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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.

Next: Etiology

Etiology

Sun exposure and/or therapeutic radiation that have caused significant skin damage are associated with the development of atypical fibroxanthoma (AFX). The tumor primarily occurs on the head or neck of older individuals.^[1]

Risk factors for the development of AFX include the use of hydrochlorothiazide (HCTZ) and diabetes mellitus.^[2]

Next: Etiology

Epidemiology

Frequency

A study in Germany of 255 patients with cutaneous sarcomas found atypical fibroxanthoma (AFX) was the most common subtype identified in nearly 50% of patients. The study also identified a significant increase in diagnoses from 2013-2019 which was attributed to the establishment of diagnostic standards leading the researchers to conclude that AFX is more common than previously assumed.^[3]

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 Presentation

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Kuntz T, Grimm A, Hofmann SC, Michalowitz AL, Schaller J, Hellmich M, et al. Hydrochlorothiazide and increased risk of atypical fibroxanthoma and pleomorphic dermal sarcoma. J Dtsch Dermatol Ges. 2024 Apr. 22 (4):513-519. [QxMD MEDLINE Link].
Kuntz T, Siebdrath J, Hofmann SC, Baltaci M, Schaller J, Hellmich M, et al. Increase of atypical fibroxanthoma and pleomorphic dermal sarcoma: a retrospective analysis of four German skin cancer centers. J Dtsch Dermatol Ges. 2022 Dec. 20 (12):1581-1588. [QxMD MEDLINE Link].
Hussein MR. Atypical fibroxanthoma: new insights. Expert Rev Anticancer Ther. 2014 Sep. 14(9):1075-88. [QxMD MEDLINE Link].
Diaz-Cascajo C, Borghi S, Bonczkowitz M. Pigmented atypical fibroxanthoma. Histopathology. 1998 Dec. 33(6):537-41. [QxMD MEDLINE Link].
Requena L, Sangueza OP, Sanchez Yus E, Furio V. Clear-cell atypical fibroxanthoma: an uncommon histopathologic variant of atypical fibroxanthoma. J Cutan Pathol. 1997 Mar. 24(3):176-82. [QxMD MEDLINE Link].
Bedir R, Agirbas S, Sehitoglu I, Yurdakul C, Elmas O. Clear cell atypical fibroxantoma: a rare variant of atypical fibroxanthoma and review of the literature. J Clin Diagn Res. 2014 Jun. 8(6):FD09-11. [QxMD MEDLINE Link]. [Full Text].
Nemejcová K, Dundr P. Granular cell variant of atypical fibroxanthoma. A case report. Cesk Patol. 2014 Winter. 50(1):34-37. [QxMD MEDLINE Link].
Rudisaile SN, Hurt MA, Santa Cruz DJ. Granular cell atypical fibroxanthoma. J Cutan Pathol. 2005 Apr. 32(4):314-7. [QxMD MEDLINE Link].
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. 1977 Aug. 97(2):167-77. [QxMD MEDLINE Link].
Singh M, Mann R, Ilankovan V, Hussein K, D'arrigo C. Atypical Fibroxanthoma-A Retrospective Immunohistochemical Study of 42 Cases. J Oral Maxillofac Surg. 2012 Apr 9. [QxMD MEDLINE Link].
Ørholt M, Abebe K, Aaberg F, Rasmussen LE, Daugaard S, Loya AC, et al. Immunohistochemical Characteristics of Atypical Fibroxanthoma and Pleomorphic Dermal Sarcoma: A Systematic Review and Meta-Analysis. Am J Dermatopathol. 2022 Dec 1. 44 (12):913-920. [QxMD MEDLINE Link].
Michie BA, Reid RP, Fallowfield ME. Aneuploidy in atypical fibroxanthoma: DNA content quantification of 10 cases by image analysis. J Cutan Pathol. 1994 Oct. 21(5):404-7. [QxMD MEDLINE Link].
Worrell JT, Ansari MQ, Ansari SJ, Cockerell CJ. Atypical fibroxanthoma: DNA ploidy analysis of 14 cases with possible histogenetic implications. J Cutan Pathol. 1993 Jun. 20(3):211-5. [QxMD MEDLINE Link].
Bugatti L, Filosa G. Dermatoscopic features of cutaneous atypical fibroxanthoma: three cases. Clin Exp Dermatol. 2009 Dec. 34(8):e898-900. [QxMD MEDLINE Link].
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. 1993 Mar. 22(3):247-54. [QxMD MEDLINE Link].
Giuffrida TJ, Kligora CJ, Goldstein GD. Localized cutaneous metastases from an atypical fibroxanthoma. Dermatol Surg. 2004 Dec. 30(12 Pt 2):1561-4. [QxMD MEDLINE Link].
Grosso M, Lentini M, Carrozza G, Catalano A. Metastatic atypical fibroxanthoma of skin. Pathol Res Pract. 1987 Jun. 182(3):443-7. [QxMD MEDLINE Link].
McClure E, Carr MJ, Patel A, Hussnain Naqvi SM, Kim Y, Harrington M, et al. Atypical Fibroxanthoma: Outcomes from a Large Single Institution Series. Cancer Control. 2023 Jan-Dec. 30:10732748231155699. [QxMD MEDLINE Link].
Polcz MM, Sebaratnam DF, Fernández-Peñas P. Atypical fibroxanthoma management: Recurrence, metastasis and disease-specific death. Australas J Dermatol. 2017 Aug 17. [QxMD MEDLINE Link].
Zalla MJ, Randle HW, Brodland DG, et al. Mohs surgery vs wide excision for atypical fibroxanthoma: follow-up. Dermatol Surg. 1997 Dec. 23(12):1223-4. [QxMD MEDLINE Link].
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. 2009 May. 35(5):765-72. [QxMD MEDLINE Link].
Fish FS. Soft tissue sarcomas in dermatology. Dermatol Surg. 1996 Mar. 22(3):268-73. [QxMD MEDLINE Link].
Phelan PS, Rosman IS, Council ML. Atypical Fibroxanthoma: The Washington University Experience. Dermatol Surg. 2019 Dec. 45 (12):1450-1458. [QxMD MEDLINE Link].
Dokic Y, Nguyen QL, Orengo I. Mohs micrographic surgery: a treatment method for many non-melanocytic skin cancers. Dermatol Online J. 2020 Apr 15. 26 (4):[QxMD MEDLINE Link].

Media Gallery

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.

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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

Richard P Vinson, MD Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster 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, Texas Medical Association, Association of Military Dermatologists, Texas Dermatological Society

Disclosure: Nothing to disclose.

Rosalie Elenitsas, MD Herman Beerman 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, American Medical Association, American Society of Dermatopathology, Pennsylvania Academy of Dermatology

Disclosure: Received royalty from Lippincott Williams Wilkins for textbook editor.

Chief Editor

William D James, MD Emeritus Professor, Department of Dermatology, University of Pennsylvania School of Medicine

William D James, MD is a member of the following medical societies: American Academy of Dermatology, American Contact Dermatitis Society, Association of Military Dermatologists, Association of Professors of Dermatology, American Dermatological Association, Women's Dermatologic Society, Medical Dermatology Society, Dermatology Foundation, Society for Investigative Dermatology, Washington DC Dermatological Society, Atlantic Dermatologic Society, Philadelphia Dermatological Society, Pennsylvania Academy of Dermatology, College of Physicians of Philadelphia

Disclosure: Received income in an amount equal to or greater than $250 from: Elsevier<br/>Served as a speaker for various universities, dermatology societies, and dermatology departments.

Additional Contributors

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.

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Atypical Fibroxanthoma

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