Angiomatoid fibrous histiocytoma of the pulmonary artery: a multidisciplinary discussion. Issue 2 (19th May 2014)

• Record Type: Journal Article
• Authors: Ghigna, Maria‐Rosa; Hamdi, Sarah; Petitpretz, Patrick; Rohnean, Adela; Florea, Valentina; Mussot, Sacha; Dartevelle, Philippe; Dorfmuller, Peter; Tu, Ly; Thuillet, Raphaël; Guignabert, Christophe; Thomas‐de‐Montpreville, Vincent
• Is Part Of: Histopathology. Volume 65:Issue 2(2014)
• Page Start: 278
• Page End: 282
• Publication Date: 2014-05-19
• Subjects: Histology, Pathological -- Periodicals; 611.018
• DOI: 10.1111/his.12429
• Languages: English
• ISSNs: 0309-0167

Abstract

<abstract abstract-type="main" id="his12429-abs-0001"> <title> <x xml:space="preserve">Abstract</x> </title> <sec id="his12429-sec-0001" sec-type="section"> <title>Aims</title> <p>Angiomatoid fibrous histiocytoma (AFH) is a rare neoplastic disease usually occurring in the dermis or subcutis of the extremities of young adults or children. Although sporadic cases in deep soft tissue and visceral organs have been reported, we present here the first description of AFH developing in a large artery.</p> </sec> <sec id="his12429-sec-0002" sec-type="section"> <title>Methods and results</title> <p>Paraffin sections of the surgical specimen were stained with haematoxylin and eosin, and immunohistochemistry was performed (CKAE1/AE3, EMA, CD34, p63, CD38, smooth muscle actin, and desmin). In addition, FISH and RT‐PCR were applied in order to check for <italic>EWRS</italic> rearrangement. The histomorphological features, and FISH analysis revealing rearrangement of <italic>EWSR, </italic> indicated the definitive diagnosis of AFH. RT‐PCR confirmed <italic>EWSR</italic> rearrangement, and detected an <italic>EWSR1–ATF1</italic> fusion transcript.</p> </sec> <sec id="his12429-sec-0003" sec-type="section"> <title>Conclusions</title> <p>A thoracic location of AFH has not been reported until very recently, and shares a differential diagnosis with diverse neoplasms, including spindle cell carcinoma and low‐grade sarcoma. We describe the first reported case of thoracic AFH arising in a large vessel, and highlight distinctive histological and molecular features.</p> </sec> </abstract>