Official reprint from UpToDate®
www.uptodate.com ©2018 UpToDate, Inc. and/or its affiliates. All Rights Reserved.

Atypical fibroxanthoma

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


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, pleomorphic dermal sarcoma, and undifferentiated pleomorphic sarcoma (UPS), a soft tissue neoplasm that shares many histologic features with AFX, is unclear [2]. While some authors consider AFX a less aggressive, superficial variant of pleomorphic dermal sarcoma and 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".)


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 [3].

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:

Subscribers log in here

Literature review current through: Dec 2017. | This topic last updated: Oct 18, 2017.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2018 UpToDate, Inc.
  1. Ziemer M. Atypical fibroxanthoma. J Dtsch Dermatol Ges 2012; 10:537.
  2. Soleymani T, Tyler Hollmig S. Conception and Management of a Poorly Understood Spectrum of Dermatologic Neoplasms: Atypical Fibroxanthoma, Pleomorphic Dermal Sarcoma, and Undifferentiated Pleomorphic Sarcoma. Curr Treat Options Oncol 2017; 18:50.
  3. Anderson HL, Joseph AK. A pilot feasibility study of a rare skin tumor database. Dermatol Surg 2007; 33:693.
  4. Wollina U, Schönlebe J, Ziemer M, et al. Atypical fibroxanthoma: a series of 56 tumors and an unexplained uneven distribution of cases in southeast Germany. Head Neck 2015; 37:829.
  5. Ang GC, Roenigk RK, Otley CC, et al. More than 2 decades of treating atypical fibroxanthoma at mayo clinic: what have we learned from 91 patients? Dermatol Surg 2009; 35:765.
  6. Fretzin DF, Helwig EB. Atypical fibroxanthoma of the skin. A clinicopathologic study of 140 cases. Cancer 1973; 31:1541.
  7. Seavolt M, McCall M. Atypical fibroxanthoma: review of the literature and summary of 13 patients treated with mohs micrographic surgery. Dermatol Surg 2006; 32:435.
  8. Mirza B, Weedon D. Atypical fibroxanthoma: a clinicopathological study of 89 cases. Australas J Dermatol 2005; 46:235.
  9. Wollina U, Schönlebe J, Koch A, Haroske G. Atypical fibroxanthoma: a series of 25 cases. J Eur Acad Dermatol Venereol 2010; 24:943.
  10. Sakamoto A, Oda Y, Itakura E, et al. Immunoexpression of ultraviolet photoproducts and p53 mutation analysis in atypical fibroxanthoma and superficial malignant fibrous histiocytoma. Mod Pathol 2001; 14:581.
  11. Dei Tos AP, Maestro R, Doglioni C, et al. Ultraviolet-induced p53 mutations in atypical fibroxanthoma. Am J Pathol 1994; 145:11.
  12. Youssef N, Vabres P, Buisson T, et al. Two unusual tumors in a patient with xeroderma pigmentosum: atypical fibroxanthoma and basosquamous carcinoma. J Cutan Pathol 1999; 26:430.
  13. Shao L, Newell B, Quintanilla N. Atypical fibroxanthoma and squamous cell carcinoma of the conjunctiva in xeroderma pigmentosum. Pediatr Dev Pathol 2007; 10:149.
  14. Dilek FH, Akpolat N, Metin A, Ugras S. Atypical fibroxanthoma of the skin and the lower lip in xeroderma pigmentosum. Br J Dermatol 2000; 143:618.
  15. Gru AA, Santa Cruz DJ. Atypical fibroxanthoma: a selective review. Semin Diagn Pathol 2013; 30:4.
  16. Hafner J, Kunzi W, Weinreich T. Malignant fibrous histiocytoma and atypical fibroxanthoma in renal transplant recipients. Dermatology 1999; 198:29.
  17. Paquet P, Piérard GE. Invasive atypical fibroxanthoma and eruptive actinic keratoses in a heart transplant patient. Dermatology 1996; 192:411.
  18. Iorizzo LJ 3rd, Brown MD. Atypical fibroxanthoma: a review of the literature. Dermatol Surg 2011; 37:146.
  19. Stadler FJ, Scott GA, Brown MD. Malignant fibrous tumors. Semin Cutan Med Surg 1998; 17:141.
  20. Diaz-Cascajo C, Borghi S, Bonczkowitz M. Pigmented atypical fibroxanthoma. Histopathology 1998; 33:537.
  21. Diaz-Cascajo C, Weyers W, Borghi S. Pigmented atypical fibroxanthoma: a tumor that may be easily mistaken for malignant melanoma. Am J Dermatopathol 2003; 25:1.
  22. Kim UR, Arora V, Shanti R, Shah AD. Neglected giant atypical fibroxanthoma of the eyelid. Ophthal Plast Reconstr Surg 2009; 25:408.
  23. Engelbrecht NE, Ford JG, White WL, Yeatts RP. Combined intraepithelial squamous neoplasia and atypical fibroxanthoma of the cornea and limbus. Am J Ophthalmol 2000; 129:94.
  24. Weedon D. Tumors and tumor-like proliferations of fibrous and related tissues. In: Weedon's Skin Pathology, 3rd ed, Elsevier Limited, 2010. p.809.
  25. Tardío JC, Pinedo F, Aramburu JA, et al. Clear Cell Atypical Fibroxanthoma: Clinicopathological Study of 6 Cases and Review of the Literature With Special Emphasis on the Differential Diagnosis. Am J Dermatopathol 2016; 38:586.
  26. Nguyen CM, Chong K, Cassarino D. Clear cell atypical fibroxanthoma: a case report and review of the literature. J Cutan Pathol 2016; 43:538.
  27. Miller K, Goodlad JR, Brenn T. Pleomorphic dermal sarcoma: adverse histologic features predict aggressive behavior and allow distinction from atypical fibroxanthoma. Am J Surg Pathol 2012; 36:1317.
  28. Morgan MB, Purohit C, Anglin TR. Immunohistochemical distinction of cutaneous spindle cell carcinoma. Am J Dermatopathol 2008; 30:228.
  29. 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 1988; 10:9.
  30. Gleason BC, Calder KB, Cibull TL, et al. Utility of p63 in the differential diagnosis of atypical fibroxanthoma and spindle cell squamous cell carcinoma. J Cutan Pathol 2009; 36:543.
  31. Ma CK, Zarbo RJ, Gown AM. Immunohistochemical characterization of atypical fibroxanthoma and dermatofibrosarcoma protuberans. Am J Clin Pathol 1992; 97:478.
  32. Hultgren TL, DiMaio DJ. Immunohistochemical staining of CD10 in atypical fibroxanthomas. J Cutan Pathol 2007; 34:415.
  33. Weedon D, Williamson R, Mirza B. CD10, a useful marker for atypical fibroxanthomas. Am J Dermatopathol 2005; 27:181.
  34. Tallon B, Beer TW. MITF positivity in atypical fibroxanthoma: a diagnostic pitfall. Am J Dermatopathol 2014; 36:888.
  35. Alomari AK, Glusac EJ, McNiff JM. p40 is a more specific marker than p63 for cutaneous poorly differentiated squamous cell carcinoma. J Cutan Pathol 2014; 41:839.
  36. Henderson SA, Torres-Cabala CA, Curry JL, et al. p40 is more specific than p63 for the distinction of atypical fibroxanthoma from other cutaneous spindle cell malignancies. Am J Surg Pathol 2014; 38:1102.
  37. Bugatti L, Filosa G. Dermatoscopic features of cutaneous atypical fibroxanthoma: three cases. Clin Exp Dermatol 2009; 34:e898.
  38. Withers AH, Brougham ND, Barber RM, Tan ST. Atypical fibroxanthoma and malignant fibrous histiocytoma. J Plast Reconstr Aesthet Surg 2011; 64:e273.
  39. Fletcher CD. The evolving classification of soft tissue tumours: an update based on the new WHO classification. Histopathology 2006; 48:3.
  40. Sakamoto A, Oda Y, Itakura E, et al. H-, K-, and N-ras gene mutation in atypical fibroxanthoma and malignant fibrous histiocytoma. Hum Pathol 2001; 32:1225.
  41. Mihic-Probst D, Zhao J, Saremaslani P, et al. CGH analysis shows genetic similarities and differences in atypical fibroxanthoma and undifferentiated high grade pleomorphic sarcoma. Anticancer Res 2004; 24:19.
  42. Love WE, Schmitt AR, Bordeaux JS. Management of unusual cutaneous malignancies: atypical fibroxanthoma, malignant fibrous histiocytoma, sebaceous carcinoma, extramammary Paget disease. Dermatol Clin 2011; 29:201.
  43. Limmer BL, Clark DP. Cutaneous micrographic surgery for atypical fibroxanthoma. Dermatol Surg 1997; 23:553.
  44. Davis JL, Randle HW, Zalla MJ, et al. A comparison of Mohs micrographic surgery and wide excision for the treatment of atypical fibroxanthoma. Dermatol Surg 1997; 23:105.
  45. Huether MJ, Zitelli JA, Brodland DG. Mohs micrographic surgery for the treatment of spindle cell tumors of the skin. J Am Acad Dermatol 2001; 44:656.
  46. Clayton BD, Leshin B, Hitchcock MG, et al. Utility of rush paraffin-embedded tangential sections in the management of cutaneous neoplasms. Dermatol Surg 2000; 26:671.
  47. McCoppin HH, Christiansen D, Stasko T, et al. Clinical spectrum of atypical fibroxanthoma and undifferentiated pleomorphic sarcoma in solid organ transplant recipients: a collective experience. Dermatol Surg 2012; 38:230.
  48. Cooper JZ, Newman SR, Scott GA, Brown MD. Metastasizing atypical fibroxanthoma (cutaneous malignant histiocytoma): report of five cases. Dermatol Surg 2005; 31:221.
  49. Helwig EB, May D. Atypical fibroxanthoma of the skin with metastasis. Cancer 1986; 57:368.
  50. Koch M, Freundl AJ, Agaimy A, et al. Atypical Fibroxanthoma - Histological Diagnosis, Immunohistochemical Markers and Concepts of Therapy. Anticancer Res 2015; 35:5717.
  51. Wang WL, Torres-Cabala C, Curry JL, et al. Metastatic atypical fibroxanthoma: a series of 11 cases including with minimal and no subcutaneous involvement. Am J Dermatopathol 2015; 37:455.
  52. Zschoche C, Hamsch C, Kutzner H, et al. Analysis of the lymphatic vessel architecture of atypical fibroxanthoma and pleomorphic dermal sarcoma. J Am Acad Dermatol 2014; 71:842.