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


RESULTS
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 EWRS rearrangement. The histomorphological features, and FISH analysis revealing rearrangement of EWSR, indicated the definitive diagnosis of AFH. RT-PCR confirmed EWSR rearrangement, and detected an EWSR1-ATF1 fusion transcript.


CONCLUSIONS
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.
Department of Pathology, Marie Lannelongue Surgical Centre, Le Plessis-Robinson, France; INSERM UMR 999, LabEx LERMIT, Marie Lannelongue Surgical Centre, Le Plessis-Robinson, France; School of Medicine, University Paris-Sud, Kremlin-Bicêtre, France.
Maria-Rosa
MR
Ghigna
Sarah
S
Hamdi
Patrick
P
Petitpretz
Adela
A
Rohnean
Valentina
V
Florea
Sacha
S
Mussot
Philippe
P
Dartevelle
Peter
P
Dorfmuller
Ly
L
Tu
Raphaël
R
Thuillet
Christophe
C
Guignabert
Vincent
V
Thomas-de-Montpreville
D000368:Aged; D014408:Biomarkers, Tumor / Q000032:analysis; D005260:Female; D051677:Histiocytoma, Malignant Fibrous / Q000235:genetics / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D017404:In Situ Hybridization, Fluorescence; D015514:Oncogene Proteins, Fusion / Q000235:genetics; D011651:Pulmonary Artery / Q000473:pathology*; D020133:Reverse Transcriptase Polymerase Chain Reaction
D014408:Biomarkers, Tumor; C560860:EWSR1-ATF1 fusion protein, human; D015514:Oncogene Proteins, Fusion
angiomatoid fibrous histiocytoma; molecular pathology; pulmonary circulation; tumour