Angiomatoid fibrous histiocytoma – case series with emphasis on a late fibrotic variant. (May 2015)

• Record Type: Journal Article
• Authors: Wilk, Michael; Zelger, Bettina G.; Debiec‐Rychter, Maria; Sciot, Raf; Zelger, Bernhard
• Is Part Of: Journal der Deutschen Dermatologischen Gesellschaft. Volume 13:Number 5(2015)
• Page Start: 441
• Page End: 448
• Publication Date: 2015-05
• Subjects: Skin -- Diseases -- Periodicals; Dermatology -- Periodicals; 616.5005
• DOI: 10.1111/ddg.12600
• Languages: English
• ISSNs: 1610-0379

Abstract

<abstract abstract-type="main"> <title>Summary</title> <sec id="ddg12600-sec-0010" sec-type="section"> <title>Background</title> <p>Angiomatoid fibrous histiocytoma (AFH) is a rare, low‐grade malignant, subcutaneous neoplasm in children or young adults.</p> </sec> <sec id="ddg12600-sec-0020" sec-type="section"> <title>Methods</title> <p>AFHs in different disease stages were studied histologically, in part, also immunohistologically, and by fluorescence in situ hybridization.</p> </sec> <sec id="ddg12600-sec-0030" sec-type="section"> <title>Results</title> <p>Depending on the degree of fibrosclerosis, nine AFH were divided into the following categories: <italic>classic type</italic> (n = 3): well‐defined subcutaneous lesions composed of multinodular spindle to epithelioid (histiocytoid) cells surrounding a pseudoangiomatous space filled with blood. Peripherally, there is a fibrous pseudocapsule and an inflammatory infiltrate. <italic>Early sclerotic type</italic> (n = 4): the fibrous capsule extends more to the inner circle of the lesion, focally replacing the cellular neoplastic component and pseudoangiomatous spaces. <italic>Late sclerotic type</italic> (n = 2): the architecture of AFH with its zonal arrangement of an outer fibrous and inner cellular component is largely replaced by fibrosis occluding the pseudovascular space in the center of the lesion. Immunohistochemistry was available in 5/9 cases with positivity for EMA (5/5), desmin (3/5), caldesmon (1/2), and CD99 (2/5). One of two cases tested displayed <italic>EWSR1</italic> rearrangement.</p> </sec> <sec id="ddg12600-sec-0040" sec-type="section"> <title>Conclusion</title> <p>Late‐stage AFH may present with marked fibrosis obscuring the real nature of the lesion and may easily be misinterpreted by the unwary as a harmless fibrotic condition.</p> </sec> </abstract>