:Atypical fibroxanthoma

Atypical fibroxanthoma (AFX) often manifests as a single, hard, pink or red papule or nodule that swells over several months and has the potential to bleed or ulcerate.{{cite journal | last=Ziemer | first=Mirjana | title=Atypical fibroxanthoma | journal=JDDG: Journal der Deutschen Dermatologischen Gesellschaft | publisher=Wiley | volume=10 | issue=8 | date=2012-06-19 | issn=1610-0379 | doi=10.1111/j.1610-0387.2012.07980.x | pages=537–548| pmid=22709412 }}{{cite journal | last1=Stadler | first1=Franz J. | last2=Scott | first2=Glynis A. | last3=Brown | first3=Marc D. | title=Malignant fibrous tumors | journal=Seminars in Cutaneous Medicine and Surgery | publisher=Frontline Medical Communications, Inc. | volume=17 | issue=2 | year=1998 | issn=1085-5629 | doi=10.1016/s1085-5629(98)80007-2 | pages=141–152| pmid=9669607 }} Tumors typically have a diameter of less than 2 cm, while they can occasionally be as large as several centimeters.{{cite journal | last1=DF | first1=Fretzin | last2=EB | first2=Helwig | title=Atypical fibroxanthoma of the skin. A clinicopathologic study of 140 cases | journal=Cancer | date=1973 | volume=31 | issue=6 | pages=1541–1552 | issn=0008-543X | pmid=4709969 | doi=10.1002/1097-0142(197306)31:6<1541::aid-cncr2820310635>3.0.co;2-6 |url=https://pubmed.ncbi.nlm.nih.gov/4709969/ | access-date=2024-04-17 }} There have been reports of an uncommon pigmented type of AFX.{{cite journal | title=Pigmented atypical fibroxanthoma | journal=Histopathology | publisher=Wiley | volume=33 | issue=6 | year=1998 | issn=0309-0167 | doi=10.1046/j.1365-2559.1998.00567.x | pages=537–541 | last1=Diaz-Cascajo | last2=Borghi | last3=Bonczkowitz | pmid=9870148 }}{{cite journal | last1=Diaz-Cascajo | first1=Carlos | last2=Weyers | first2=Wolfgang | last3=Borghi | first3=Susanna | title=Pigmented Atypical Fibroxanthoma | journal=The American Journal of Dermatopathology | publisher=Ovid Technologies (Wolters Kluwer Health) | volume=25 | issue=1 | year=2003 | issn=0193-1091 | doi=10.1097/00000372-200302000-00001 | pages=1–5}}

The most frequent locations for AFX are the head and neck. Less frequently occurring tumors of the trunk and extremities may appear as bigger tumors. Sites like the cornea or eyelid are rarely impacted.{{cite journal | last1=Kim | first1=Usha R. | last2=Arora | first2=Vipul | last3=Shanti | first3=R | last4=Shah | first4=Akash D. | title=Neglected Giant Atypical Fibroxanthoma of the Eyelid | journal=Ophthalmic Plastic & Reconstructive Surgery | publisher=Ovid Technologies (Wolters Kluwer Health) | volume=25 | issue=5 | year=2009 | issn=0740-9303 | doi=10.1097/iop.0b013e3181b399c0 | pages=408–409}}{{cite journal | last1=Engelbrecht | first1=Nicholas E | last2=Ford | first2=Jerry G | last3=White | first3=Wain L | last4=Yeatts | first4=R.Patrick | title=Combined intraepithelial squamous neoplasia and atypical fibroxanthoma of the cornea and limbus | journal=American Journal of Ophthalmology | publisher=Elsevier BV | volume=129 | issue=1 | year=2000 | issn=0002-9394 | doi=10.1016/s0002-9394(99)00275-5 | pages=94–96| pmid=10653421 }}

The majority of AFX tumors don't cause any symptoms. Rarely the lesion may be tender. It is rare to experience pruritus.

It is unclear what causes atypical fibroxanthomas. It is thought to originate from myofibroblasts or cells that resemble fibroblasts.{{cite journal | last1=IORIZZO | first1=LUCIANO J. | last2=BROWN | first2=MARC D. | title=Atypical Fibroxanthoma: A Review of the Literature | journal=Dermatologic Surgery | publisher=Ovid Technologies (Wolters Kluwer Health) | volume=37 | issue=2 | year=2011 | issn=1076-0512 | doi=10.1111/j.1524-4725.2010.01843.x | pages=146–157| pmid=21269345 }} Since the majority of lesions form on White patients' sun-exposed heads and necks, ultraviolet light appears to be important.{{cite journal | last1=Ang | first1=Gina C. | last2=Roenigk | first2=Randall K. | last3=Otley | first3=Clark C. | last4=Phillips | first4=Kim P. | last5=Weaver | first5=Amy L. | title=More Than 2 Decades of Treating Atypical Fibroxanthoma at Mayo Clinic | journal=Dermatologic Surgery | publisher=Ovid Technologies (Wolters Kluwer Health) | volume=35 | issue=5 | year=2009 | issn=1076-0512 | doi=10.1111/j.1524-4725.2009.01126.x | pages=765–772| pmid=19389106 }}

In AFX, P53 mutations as well as cyclobutane pyrimidine dimers, UV photoproducts implicated in the development of skin cancer, have been found.{{cite journal | last1=Sakamoto | first1=Akio | last2=Oda | first2=Yoshinao | last3=Itakura | first3=Eijun | last4=Oshiro | first4=Yumi | last5=Nikaido | first5=Osamu | last6=Iwamoto | first6=Yukihide | last7=Tsuneyoshi | first7=Masazumi | title=Immunoexpression of Ultraviolet Photoproducts and p53 Mutation Analysis in Atypical Fibroxanthoma and Superficial Malignant Fibrous Histiocytoma | journal=Modern Pathology | publisher=Elsevier BV | volume=14 | issue=6 | year=2001 | issn=0893-3952 | doi=10.1038/modpathol.3880354 | pages=581–588| pmid=11406660 | doi-access=free }}{{cite journal | last1=AP | first1=Dei Tos | last2=R | first2=Maestro | last3=C | first3=Doglioni | last4=D | first4=Gasparotto | last5=M | first5=Boiocchi | last6=L | first6=Laurino | last7=CD | first7=Fletcher | title=Ultraviolet-induced p53 mutations in atypical fibroxanthoma | journal=The American Journal of Pathology | date=1994 | publisher=Am J Pathol | volume=145 | issue=1 | pages=11–17 | issn=0002-9440 | pmid=8030743 | pmc=1887293 }} AFX has been observed in children with xeroderma pigmentosum.{{cite journal | last1=Youssef | first1=N. | last2=Vabres | first2=P. | last3=Buisson | first3=T. | last4=Brousse | first4=N. | last5=Fraitag | first5=S. | title=Two unusual tumors in a patient with xeroderma pigmentosum: atypical fibroxanthoma and basosquamous carcinoma | journal=Journal of Cutaneous Pathology | publisher=Wiley | volume=26 | issue=9 | year=1999 | issn=0303-6987 | doi=10.1111/j.1600-0560.1999.tb01870.x | pages=430–435| pmid=10563498 }}{{cite journal | last1=Shao | first1=Lei | last2=Newell | first2=Brandon | last3=Quintanilla | first3=Norma | title=Atypical Fibroxanthoma and Squamous Cell Carcinoma of the Conjunctiva in Xeroderma Pigmentosum | journal=Pediatric and Developmental Pathology | publisher=SAGE Publications | volume=10 | issue=2 | year=2007 | issn=1093-5266 | doi=10.2350/06-06-0103.1 | pages=149–152| pmid=17378688 }} There have been other hypothesized causes of AFX, such as radiation therapy, immunosuppression,{{cite journal | last1=Paquet | first1=P. | last2=Piérard | first2=G.E. | title=Invasive Atypical Fibroxanthoma and Eruptive Actinic Keratoses in a Heart Transplant Patient | journal=Dermatology | publisher=S. Karger AG | volume=192 | issue=4 | year=1996 | issn=1018-8665 | doi=10.1159/000246435 | pages=411–413| pmid=8864396 }} burns,{{cite journal | last1=Hiscutt | first1=E.L. | last2=Adams | first2=J.R. | last3=Ryan | first3=J.M. | last4=Langtry | first4=J.A.A. | last5=Natarajan | first5=S. | title=Atypical fibroxanthoma, lentigo maligna melanoma and squamous cell carcinoma arising in the site of a thermal burn treated with skin grafts | journal=British Journal of Oral and Maxillofacial Surgery | publisher=Elsevier BV | volume=47 | issue=2 | year=2009 | issn=0266-4356 | doi=10.1016/j.bjoms.2008.08.017 | pages=157–158| pmid=18963287 }} and trauma.{{cite journal | last1=Gru | first1=Alejandro A. | last2=Santa Cruz | first2=Daniel J. | title=Atypical fibroxanthoma: a selective review | journal=Seminars in Diagnostic Pathology | publisher=Elsevier BV | volume=30 | issue=1 | year=2013 | issn=0740-2570 | doi=10.1053/j.semdp.2012.01.001 | pages=4–12| pmid=23327726 }}

Since atypical fibroxanthoma is an uncommon disorder that mimics amelanotic melanomas, Merkel cell carcinomas, and basal and squamous cell carcinomas clinically, a skin biopsy is the gold standard for diagnosis.{{cite web | last1=Kolb | first1=Logan | last2=Schmieder | first2=George J. | title=Atypical Fibroxanthoma | publisher=StatPearls Publishing | date=2022-09-18 | pmid=29083667 |url=https://www.ncbi.nlm.nih.gov/books/NBK459342/ | access-date=2024-04-17}}

Atypical fibroxanthoma (AFX) is frequently found to have a well-circumscribed, nonencapsulated dermal tumor that is either contiguous with the epidermis or separated from it by a narrow zone of collagen (Grenz zone); plump spindle cells with prominent nuclei, epithelioid cells, and multinucleated giant cells; atypical mitoses and severe cellular pleomorphism; and varying presence of thin or ulcerated epidermis or peripheral epidermal collarette.

For atypical fibroxanthoma, surgical excision is the preferred course of treatment. This was done with 1 cm margins in the past, but Mohs micrographic surgery and routine follow-up have become the norm, with recurrence rates ranging from 0.0% to 6.9%.

Men are more likely to experience AFX than women, and it often affects adults in their seventh or eighth decade of life.{{cite journal | last1=Wollina | first1=Uwe | last2=Schönlebe | first2=Jaqueline | last3=Ziemer | first3=Mirjana | last4=Friedling | first4=Franziska | last5=Koch | first5=André | last6=Haroske | first6=Gunter | last7=Kaatz | first7=Martin | last8=Simon | first8=Jan-C. | title=Atypical fibroxanthoma: A series of 56 tumors and an unexplained uneven distribution of cases in southeast Germany | journal=Head & Neck | publisher=Wiley | volume=37 | issue=6 | date=2014-06-20 | issn=1043-3074 | doi=10.1002/hed.23673 | pages=829–834| pmid=24946737 }} While AFX can affect people of any ethnicity, non-Hispanic White people have been the majority of those with reported instances. Patients with AFX frequently have a personal history of previous basal cell or squamous cell cancer.{{cite journal | last1=SEAVOLT | first1=MARALYN | last2=MCCALL | first2=MICHAEL | title=Atypical Fibroxanthoma: Review of the Literature and Summary of 13 Patients Treated with Mohs Micrographic Surgery | journal=Dermatologic Surgery | publisher=Ovid Technologies (Wolters Kluwer Health) | volume=32 | issue=3 | date=2006-03-31 | issn=1076-0512 | doi=10.1111/j.1524-4725.2006.32087.x | pages=435–441| pmid=16640694 }}

• Skin lesion
• Skin cancer

{{reflist}}

• {{cite journal | last=Sakamoto | first=Akio | title=Atypical Fibroxanthoma | journal=Clinical Medicine. Oncology | publisher=SAGE Publications | volume=2 | year=2008 | pages=117–127 | issn=1177-9314 | doi=10.4137/cmo.s506 | pmid=21892274 | pmc=3161641 | ref=none}}
• {{cite journal | last1=Mirza | first1=Basit | last2=Weedon | first2=David | title=Atypical fibroxanthoma: A clinicopathological study of 89 cases | journal=Australasian Journal of Dermatology | publisher=Wiley | volume=46 | issue=4 | date=2005-09-30 | issn=0004-8380 | doi=10.1111/j.1440-0960.2005.00190.x | pages=235–238 | pmid=16197421 | ref=none}}

• [https://dermnetnz.org/topics/atypical-fibroxanthoma DermNet]
• [https://www.pathologyoutlines.com/topic/skintumornonmelanocyticAFX.html Pathology Outlines]

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{{Soft tissue tumors and sarcomas}}

Category:Dermal and subcutaneous growths