Abstract
Atypical fibroxanthoma (AFX) and malignant fibrous histiocytoma (MFH) are soft tissue fibrohistiocytic sarcomas with variable aggressiveness. There is considerable debate about the relationship between AFX and MFH, as histological and immunochemical differentiation is difficult. Some authors consider AFX as the superficial/dermal variant of MFH, now reclassified as “undifferentiated pleomorphic sarcoma (UPS).” Their different biological behavior is explained by the absence of ras-oncogene mutations in AFX, whereas MFH has both H- and K-ras gene mutations which contribute towards the less favorable prognosis of MFH. Otherwise, they have been classified as similar entities within a spectrum of fibrohistiocytic malignancies.
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Rongioletti, F. (2015). Atypical Fibroxanthoma/Undifferentiated Pleomorphic Sarcoma (Superficial Malignant Fibrous Histiocytoma). In: Rare Malignant Skin Tumors. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-2023-5_33
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DOI: https://doi.org/10.1007/978-1-4939-2023-5_33
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