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Atypical Fibroxanthoma

MedGen UID:
138033
•Concept ID:
C0346053
•
Neoplastic Process
Synonyms: Atypical Cutaneous Fibroxanthoma; Atypical fibrous histiocytoma; Atypical Fibrous Histiocytoma; Atypical fibroxanthoma; Atypical fibroxanthoma of skin; Atypical Fibroxanthoma of Skin; Atypical Fibroxanthoma of the Skin; Atypical Skin Fibroxanthoma; Fibroxanthosarcoma of skin
SNOMED CT: Atypical fibroxanthoma of skin (254754005); Fibroxanthosarcoma of skin (254754005); Atypical fibrous histiocytoma (26496005); Atypical fibroxanthoma (26496005)

Definition

An intermediate cutaneous mesenchymal neoplasm of uncertain differentiation, usually affecting the actinic-damaged skin of the elderly. The tumor presents as a single cutaneous nodule which is often ulcerated. Microscopically, it is characterized by the presence of highly atypical cytologic features, pleomorphism and abundant mitotic figures. The vast majority of patients have an excellent prognosis following conservative therapy. Recurrences are infrequent, and metastasis is rare. [from NCI]

Professional guidelines

PubMed

Management of Skin Sarcomas.
Messina V, Cope B, Keung EZ, Fiore M
Surg Oncol Clin N Am 2022 Jul;31(3):511-525. Epub 2022 May 31 doi: 10.1016/j.soc.2022.03.010. PMID: 35715147
Atypical Fibroxanthoma and Pleomorphic Dermal Sarcoma: Updates on Classification and Management.
Soleymani T, Aasi SZ, Novoa R, Hollmig ST
Dermatol Clin 2019 Jul;37(3):253-259. Epub 2019 Apr 16 doi: 10.1016/j.det.2019.02.001. PMID: 31084719
Leiomyosarcoma and Pleomorphic Dermal Sarcoma: Guidelines for Diagnosis and Treatment.
Llombart B, Serra-Guillén C, Requena C, Alsina M, Morgado-Carrasco D, Machado I, Sanmartín O
Actas Dermosifiliogr (Engl Ed) 2019 Jan-Feb;110(1):4-11. Epub 2018 Nov 5 doi: 10.1016/j.ad.2018.06.012. PMID: 30409380

Recent clinical studies

Etiology

Cutaneous Sarcomas.
Tillman BN, Liu JC
Otolaryngol Clin North Am 2021 Apr;54(2):369-378. Epub 2021 Feb 16 doi: 10.1016/j.otc.2020.11.010. PMID: 33602520
Atypical Fibroxanthoma and Pleomorphic Dermal Sarcoma: Updates on Classification and Management.
Soleymani T, Aasi SZ, Novoa R, Hollmig ST
Dermatol Clin 2019 Jul;37(3):253-259. Epub 2019 Apr 16 doi: 10.1016/j.det.2019.02.001. PMID: 31084719
Fibrohistiocytic Tumors.
Romano RC, Fritchie KJ
Clin Lab Med 2017 Sep;37(3):603-631. Epub 2017 Jun 15 doi: 10.1016/j.cll.2017.05.007. PMID: 28802503
Cutaneous sarcomas.
Kohlmeyer J, Steimle-Grauer SA, Hein R
J Dtsch Dermatol Ges 2017 Jun;15(6):630-648. doi: 10.1111/ddg.13249. PMID: 28591457
Nonmelanoma skin cancer.
Dubas LE, Ingraffea A
Facial Plast Surg Clin North Am 2013 Feb;21(1):43-53. doi: 10.1016/j.fsc.2012.10.003. PMID: 23369588

Diagnosis

Pleomorphic Dermal Sarcoma.
Saleh JS, Whittington CP, Bresler SC, Patel RM
Surg Pathol Clin 2024 Mar;17(1):153-158. Epub 2023 Aug 5 doi: 10.1016/j.path.2023.06.007. PMID: 38278604
Atypical Fibroxanthoma and Pleomorphic Dermal Sarcoma: Updates on Classification and Management.
Soleymani T, Aasi SZ, Novoa R, Hollmig ST
Dermatol Clin 2019 Jul;37(3):253-259. Epub 2019 Apr 16 doi: 10.1016/j.det.2019.02.001. PMID: 31084719
Atypical Fibroxanthoma.
Chapman LW, Yu SS, Arron ST
Semin Cutan Med Surg 2019 Mar 1;38(1):E65-E66. doi: 10.12788/j.sder.2019.008. PMID: 31051027
Fibrohistiocytic Tumors.
Romano RC, Fritchie KJ
Clin Lab Med 2017 Sep;37(3):603-631. Epub 2017 Jun 15 doi: 10.1016/j.cll.2017.05.007. PMID: 28802503
Atypical Fibroxanthoma.
López L, Vélez R
Arch Pathol Lab Med 2016 Apr;140(4):376-9. doi: 10.5858/arpa.2014-0495-RS. PMID: 27028396

Therapy

Hydrochlorothiazide and increased risk of atypical fibroxanthoma and pleomorphic dermal sarcoma.
Kuntz T, Grimm A, Hofmann SC, Michalowitz AL, Schaller J, Hellmich M, Assaf C, Oellig F, Kreuter A
J Dtsch Dermatol Ges 2024 Apr;22(4):513-519. Epub 2024 Mar 14 doi: 10.1111/ddg.15379. PMID: 38483049
Immunohistochemical Characteristics of Atypical Fibroxanthoma and Pleomorphic Dermal Sarcoma: A Systematic Review and Meta-Analysis.
Ørholt M, Abebe K, Aaberg F, Rasmussen LE, Daugaard S, Loya AC, Herly M, Vester-Glowinski PV
Am J Dermatopathol 2022 Dec 1;44(12):913-920. doi: 10.1097/DAD.0000000000002305. PMID: 36395448
Atypical Fibroxanthoma: The Washington University Experience.
Phelan PS, Rosman IS, Council ML
Dermatol Surg 2019 Dec;45(12):1450-1458. doi: 10.1097/DSS.0000000000001943. PMID: 30939522
Atypical fibroxanthoma: new insights.
Hussein MR
Expert Rev Anticancer Ther 2014 Sep;14(9):1075-88. Epub 2014 Jun 24 doi: 10.1586/14737140.2014.924401. PMID: 24958232
Fibrous and fibrohistiocytic neoplasms: an update.
Clarke LE
Dermatol Clin 2012 Oct;30(4):643-56, vi. Epub 2012 Aug 11 doi: 10.1016/j.det.2012.06.005. PMID: 23021051

Prognosis

Pleomorphic Dermal Sarcoma.
Saleh JS, Whittington CP, Bresler SC, Patel RM
Surg Pathol Clin 2024 Mar;17(1):153-158. Epub 2023 Aug 5 doi: 10.1016/j.path.2023.06.007. PMID: 38278604
Atypical Fibroxanthoma and Pleomorphic Dermal Sarcoma: Updates on Classification and Management.
Soleymani T, Aasi SZ, Novoa R, Hollmig ST
Dermatol Clin 2019 Jul;37(3):253-259. Epub 2019 Apr 16 doi: 10.1016/j.det.2019.02.001. PMID: 31084719
Cutaneous sarcomas.
Kohlmeyer J, Steimle-Grauer SA, Hein R
J Dtsch Dermatol Ges 2017 Jun;15(6):630-648. doi: 10.1111/ddg.13249. PMID: 28591457
Atypical Fibroxanthoma.
López L, Vélez R
Arch Pathol Lab Med 2016 Apr;140(4):376-9. doi: 10.5858/arpa.2014-0495-RS. PMID: 27028396
Atypical fibroxanthoma: new insights.
Hussein MR
Expert Rev Anticancer Ther 2014 Sep;14(9):1075-88. Epub 2014 Jun 24 doi: 10.1586/14737140.2014.924401. PMID: 24958232

Clinical prediction guides

Atypical fibroxanthoma and pleomorphic dermal sarcoma: A reappraisal.
Cesinaro AM, Gallo G, Tramontozzi S, Migaldi M
J Cutan Pathol 2021 Feb;48(2):207-210. Epub 2020 Jul 28 doi: 10.1111/cup.13787. PMID: 32583897
Pseudolymphomatous Atypical Fibroxanthoma.
Garcia Castro R, Viñolas-Cuadros A, Moyano-Bueno D, Conde-Ferreiros A, Cardeñoso E, Santos-Briz A
Am J Dermatopathol 2020 Dec;42(12):972-976. doi: 10.1097/DAD.0000000000001761. PMID: 32769551
Atypical Fibroxanthoma and Pleomorphic Dermal Sarcoma: Updates on Classification and Management.
Soleymani T, Aasi SZ, Novoa R, Hollmig ST
Dermatol Clin 2019 Jul;37(3):253-259. Epub 2019 Apr 16 doi: 10.1016/j.det.2019.02.001. PMID: 31084719
Atypical Fibroxanthoma - Histological Diagnosis, Immunohistochemical Markers and Concepts of Therapy.
Koch M, Freundl AJ, Agaimy A, Kiesewetter F, Künzel J, Cicha I, Alexiou C
Anticancer Res 2015 Nov;35(11):5717-35. PMID: 26503993
Atypical fibroxanthoma: new insights.
Hussein MR
Expert Rev Anticancer Ther 2014 Sep;14(9):1075-88. Epub 2014 Jun 24 doi: 10.1586/14737140.2014.924401. PMID: 24958232

Recent systematic reviews

Immunohistochemical Characteristics of Atypical Fibroxanthoma and Pleomorphic Dermal Sarcoma: A Systematic Review and Meta-Analysis.
Ørholt M, Abebe K, Aaberg F, Rasmussen LE, Daugaard S, Loya AC, Herly M, Vester-Glowinski PV
Am J Dermatopathol 2022 Dec 1;44(12):913-920. doi: 10.1097/DAD.0000000000002305. PMID: 36395448
Surgical excision margins for fibrohistiocytic tumors, including atypical fibroxanthoma and undifferentiated pleomorphic sarcoma: A probability model based on a systematic review.
Jibbe A, Worley B, Miller CH, Alam M
J Am Acad Dermatol 2022 Oct;87(4):833-840. Epub 2021 Sep 26 doi: 10.1016/j.jaad.2021.09.036. PMID: 34587553
Time to recurrence after surgical excision of atypical fibroxanthoma-updated systematic review and meta-analysis.
Phan K, Onggo J
Australas J Dermatol 2019 Aug;60(3):e220-e222. Epub 2019 Feb 21 doi: 10.1111/ajd.13010. PMID: 30793288
Atypical fibroxanthoma: Systematic review and meta-analysis of treatment with Mohs micrographic surgery or excision.
Tolkachjov SN, Kelley BF, Alahdab F, Erwin PJ, Brewer JD
J Am Acad Dermatol 2018 Nov;79(5):929-934.e6. Epub 2018 Jul 5 doi: 10.1016/j.jaad.2018.06.048. PMID: 29981390

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