Diagnostic Histopathology
Volume 16, Issue 9 , Pages 401-408, September 2010

Atypical fibroxanthoma: differential diagnosis from other sarcomatoid skin lesions

Richard A Scolyer MD FRCPA FRCPath is a Senior Staff Specialist in the Department of Tissue Pathology and Diagnostic Oncology at the Royal Prince Alfred Hospital, Camperdown, NSW, Australia; he is also Clinical Professor of Pathology at The University of Sydney Medical School, and Clinical Research Fellow at the Cancer Institute of New South Wales, Australia. Conflicts of interest: none declared

Rajmohan Murali MBBS FRCPA is Staff Specialist in the Department of Tissue Pathology and Diagnostic Oncology at the Royal Prince Alfred Hospital, Camperdown, NSW, Australia; he is also Clinical Senior Lecturer in Pathology at The University of Sydney Medical School, and Clinical Research Fellow at the Cancer Institute of New South Wales, Australia. Conflicts of interest: none declared

Stanley W McCarthy MBBS FRCPA is a Senior Staff Specialist in the Department of Tissue Pathology and Diagnostic Oncology at the Royal Prince Alfred Hospital, Camperdown, NSW, Australia; he is also a Clinical Professor in Pathology at The University of Sydney Medical School, New South Wales, Australia. Conflicts of interest: none declared

John F Thompson MD FRACS FACS is a Professor of Melanoma and Surgical Oncology at the University of Sydney Medical School and Executive Director of the Melanoma Institute of Australia. Conflicts of interest: none declared

published online 12 July 2010.

Abstract 

Atypical fibroxanthoma (AFX) is an uncommon cutaneous neoplasm that usually presents as a rapidly-growing nodule in sun-exposed sites in elderly patients. Despite its highly atypical histological appearance it is almost always associated with innocuous clinical behaviour. AFX is now generally regarded as the superficial counterpart of undifferentiated pleomorphic sarcoma (so-called malignant fibrous histiocytoma [MFH]). The former lesion is associated with an excellent prognosis in view of its small size, superficial location, and amenability to complete excision. Because a distinction between AFX and MFH requires assessment of the depth of invasion, a definitive diagnosis of AFX cannot be made on the basis of shallow biopsies. Other cutaneous tumours, including sarcomatoid squamous cell carcinoma (SCC) and spindle-cell melanoma, may have a histologic appearance that is indistinguishable from AFX on haematoxylin–eosin stained slides; immunochemical stains are therefore mandatory in the pathologic evaluation of such cases. There are no currently-known specific immunohistochemical markers (including CD10) which are diagnostic of AFX, and it remains a diagnosis of exclusion. Recent studies have highlighted the importance of other markers, such as high molecular-weight keratins (e.g., CK5/6, 34BE12, and MNF116) and p63 in the diagnosis of sarcomatoid squamous cell carcinoma; that tumour may fail to label for other keratin proteins. Recently, uncommon variants of AFX have been described that broaden its histological differential diagnosis.

Keywords: atypical fibroxanthoma, carcinoma, diagnosis, differential diagnosis, melanoma, pathology, sarcoma, skin

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PII: S1756-2317(10)00095-2

doi:10.1016/j.mpdhp.2010.06.007

Diagnostic Histopathology
Volume 16, Issue 9 , Pages 401-408, September 2010