Solitary fibrous tumour of the genitourinary tract: a clinicopathological study of 11 cases and their association with theNAB2-STAT6fusion gene.
OBJECTIVE
To characterise clinicopathological features and clinical outcomes of the genitourinary tract solitary fibrous tumours, incorporatingNAB2-STAT6gene fusion status.


METHODS
The presence of the molecular hallmarkNAB2-STAT6gene fusion and for the defining fusion partner product STAT6 was assessed in 11 cases of the genitourinary tract solitary fibrous tumours.NAB2-STAT6gene fusion analysis was performed using a break-apart fluorescence in situ hybridisation (FISH) probe using a probe cocktail with Bacterial artificial chromosome (BAC) clones forSTAT6andNAB2.


RESULTS
Eleven solitary fibrous tumours were diagnosed in eight male patients and three female patients with a mean age of 46 years (range: 11-64 years). Four of the tumours had malignant histological features, and three were considered moderate risk for metastasis. With a mean follow-up time of 61 months, 1 recurred locally and 2 presented at distant metastatic sites. Using a break-apart FISH probe cocktail, we found theNAB2-STAT6gene fusion and nuclear STAT6 expression in 58% and 91% of cases, respectively. However, theNAB2-STAT6fusion status was not correlated with STAT6 expression or useful in discriminating between malignant histological features or subsequent clinical outcomes in the genitourinary solitary fibrous tumours.


CONCLUSIONS
A subset of solitary fibrous tumours of the genitourinary tract behaved aggressively. Using a break-apart FISH probe cocktail, we found theNAB2-STAT6gene fusion in 64% of cases. However, theNAB2-STAT6fusion status was not correlated with STAT6 expression or useful in discriminating between low-risk or high-risk tumours and subsequent clinical outcomes.
Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis, Indiana, USA.
Erik
E
Kouba
Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis, Indiana, USA.
Novae B
NB
Simper
Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis, Indiana, USA.
Shaoxiong
S
Chen
Department of Pathology and Laboratory Medicine, Henry Ford Health System, Detroit, Michigan, USA.
Sean R
SR
Williamson
Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis, Indiana, USA.
David J
DJ
Grignon
Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis, Indiana, USA.
John N
JN
Eble
Departments of Pathology and Laboratory Medicine, Case Western Reserve University, Cleveland, Ohio, USA.
Gregory T
GT
MacLennan
Department of Pathological Anatomy and Histopathology, School of Medicine, Polytechnic University of the Marche Region (Ancona), Ancona, Italy.
Rodolfo
R
Montironi
Unit of Anatomical Pathology, Department of Surgery, Faculty of Medicine, Cordoba, Spain.|Champalimaud Clinical Center, Lisbon, Portugal.
Antonio
A
Lopez-Beltran
Department of Pathology, Emory University School of Medicine, Atlanta, Georgia, USA.
Adeboye O
AO
Osunkoya
Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis, Indiana, USA.
Shaobo
S
Zhang
Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis, Indiana, USA.
Mingsheng
M
Wang
Michigan Center for Translational Pathology, University of Michigan, Ann Arbor, Michigan, USA.
Lisha
L
Wang
Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis, Indiana, USA.
Thu
T
Tran
Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis, Indiana, USA.
Robert E
RE
Emerson
Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis, Indiana, USA.
Lee Ann
LA
Baldrige
Department of Urology, Indiana University School of Medicine, Indianapolis, Indiana, USA.
M Francesca
MF
Monn
Department of Pathology and Laboratory Medicine, Dartmouth-Hitchcock Medical Center, Geisel School of Medicine at Dartmouth, Hanover, New Hampshire, USA.
Konstantinos
K
Linos
Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis, Indiana, USA.|Department of Urology, Indiana University School of Medicine, Indianapolis, Indiana, USA.
Liang
L
Cheng
D000293:Adolescent; D000310:Adrenal Gland Neoplasms / Q000235:genetics* / Q000473:pathology; D000328:Adult; D002648:Child; D018450:Disease Progression; D005260:Female; D050939:Gene Fusion / Q000235:genetics; D006801:Humans; D008297:Male; D008875:Middle Aged; D012097:Repressor Proteins / Q000235:genetics*; D050800:STAT6 Transcription Factor / Q000235:genetics*; D054364:Solitary Fibrous Tumors / Q000235:genetics* / Q000473:pathology; D014565:Urogenital Neoplasms / Q000235:genetics* / Q000473:pathology; D055815:Young Adult
C491000:NAB2 protein, human; D012097:Repressor Proteins; D050800:STAT6 Transcription Factor; C494101:STAT6 protein, human
CYTOGENETICS; GENITOURINARY PATHOLOGY; IN SITU HYBRIDISATION; KIDNEY