Histopathology. 2014 Apr 4. doi: 10.1111/his.12429. [Epub ahead of print]

Angiomatoid fibrous histiocytoma of the pulmonary artery: a multidisciplinary discussion.

Ghigna MR1, Hamdi S, Petitpretz P, Rohnean A, Florea V, Mussot S, Dartevelle P, Dorfmuller P, Tu L, Thuillet R, Guignabert C, Thomas-de-Montpreville V.

Department of Pathology, Marie Lannelongue Surgical Centre, 92350, Le Plessis Robinson, France
INSERM UMR 999, LabEx LERMIT, Marie Lannelongue Surgical Centre, Univ Paris-Sud, School of medicine, Kremlin-Bicêtre, France.

Abstract

AIM: Angiomatoid fibrous histiocytoma (AFH) is a rare neoplastic disease usually occurring in dermis or subcutis of the extremities of young adults or children. Though sporadic cases in deep soft tissue and visceral organs have been reported, this is the first description of AFH developing in a large artery.
METHODS: Paraffin sections of the surgical specimen were stained by hematoxylin and eosin and immunohistochemistry was performed (CKAE1/AE3, EMA, CD34, p63, CD38, SMA, desmin). In addition FISH technique and RT-PCR were applied in order to check EWRS rearrangement.
RESULTS: Histomorphologic features and FISH analysis revealing the rearrangement of the EWSR gene, assigned the definitive diagnosis of angiomatoid fibrous histiocytoma. RT-PCR confirmed EWSR rearrangement and detected an EWSR1-ATF1 fusion transcript.
CONCLUSION: The thoracic location of AFH has not been illustrated until very recently and it shares differential diagnosis with diverse neoplasms including spindle cell carcinoma and low-grade sarcoma. Our report describes the first case of thoracic AFH arising in a large vessel and points out the distinctive histological and molecular features of this recent entity.

KEYWORDS: angiomatoid fibrous histiocytoma, molecular pathology, pulmonary circulation, tumour

PMID:24702653