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Atypical Fibroxanthoma

  • Living reference work entry
  • First Online:
Encyclopedia of Pathology

Part of the book series: Encyclopedia of Pathology ((EP))

  • 29 Accesses

Synonyms

Dermal undifferentiated pleomorphic sarcoma; Superficial “malignant fibrous histiocytoma” (obsolete)

Definition

Atypical fibroxanthoma is a pleomorphic dermal-based and nodular tumor affecting sun-damaged skin of the elderly (Mirza and Weedon 2005). The tumors are of uncertain differentiation and pursue an indolent clinical course if strict diagnostic criteria are applied. Similar tumors with additional invasion of subcutis, tumor necrosis, or perineural or lymphovascular invasion are regarded as pleomorphic dermal sarcoma due to their potential for outright malignant behavior (Miller et al. 2012).

Clinical Features

  • Incidence

    The incidence is highest in Caucasians living in areas of high UV-rates. The true worldwide incidence rates are unknown.

  • Age

    The tumors affect the elderly with an average age of 70 years (Mirza and Weedon 2005).

  • Sex

    There is a male predominance.

  • Site

    Chronically sun-damaged skin is exclusively affected with a marked predilection for the head and neck area...

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References and Further Reading

  • Beer, T. W., Drury, P., & Heenan, P. J. (2010). Atypical fibroxanthoma: A histological and immunohistochemical review of 171 cases. The American Journal of Dermatopathology, 32(6), 533–540.

    Article  Google Scholar 

  • Griewank, K. G., Wiesner, T., Murali, R., et al. (2018). Atypical fibroxanthoma and pleomorphic dermal sarcoma harbor frequent NOTCH1/2 and FAT1 mutations and similar DNA copy number alteration profiles. Modern Pathology, 31(3), 418–428.

    Article  CAS  Google Scholar 

  • Luzar, B., & Calonje, E. (2010). Morphological and immunohistochemical characteristics of atypical fibroxanthoma with a special emphasis on potential diagnostic pitfalls: A review. Journal of Cutaneous Pathology, 37(3), 301–309.

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  • Miller, K., Goodlad, J. R., & Brenn, T. (2012). Pleomorphic dermal sarcoma: Adverse histologic features predict aggressive behavior and allow distinction from atypical fibroxanthoma. The American Journal of Surgical Pathology, 36(9), 1317–1326.

    Article  Google Scholar 

  • Mirza, B., & Weedon, D. (2005). Atypical fibroxanthoma: A clinicopathological study of 89 cases. The Australasian Journal of Dermatology, 46(4), 235–238.

    Article  Google Scholar 

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Author information

Authors and Affiliations

  1. Department of Pathology and Laboratory Medicine and The Arnie Charbonneau Cancer Institute, Cumming School of Medicine, University of Calgary, Calgary, AB, Canada

    Thomas Brenn

Authors
  1. Thomas Brenn
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Corresponding author

Correspondence to Thomas Brenn .

Editor information

Editors and Affiliations

  1. NIJMEGEN, The Netherlands

    J.H.J.M. van Krieken

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Brenn, T. (2020). Atypical Fibroxanthoma. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_5353-1

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  • DOI: https://doi.org/10.1007/978-3-319-28845-1_5353-1

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-28845-1

  • Online ISBN: 978-3-319-28845-1

  • eBook Packages: Springer Reference MedicineReference Module Medicine

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