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Solitary fibrous tumour of the genitourinary tract: a clinicopathological study of 11 cases and their association with the NAB2-STAT6 fusion gene

2016-10-31 21:22:38

Journal of Clinical Pathology; 31 October 2016: DOI:10.1136/jclinpath-2016-204088

Erik Kouba, Novae B Simper, Shaoxiong Chen, Sean R Williamson, David J Grignon, John N Eble, Gregory T MacLennan, Rodolfo Montironi, Antonio Lopez-Beltran, Adeboye O Osunkoya, Shaobo Zhang, Mingsheng Wang, Lisha Wang, Thu Tran, Robert E Emerson, Lee Ann Baldrige, M Francesca Monn, Konstantinos Linos, Liang Cheng



Abstract


Aims


To characterise clinicopathological features and clinical outcomes of the genitourinary tract solitary fibrous tumours, incorporating NAB2-STAT6 gene fusion status.



Methods


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



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 the NAB2-STAT6 gene fusion and nuclear STAT6 expression in 58% and 91% of cases, respectively. However, the NAB2-STAT6 fusion 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 the NAB2-STAT6 gene fusion in 64% of cases. However, the NAB2-STAT6 fusion status was not correlated with STAT6 expression or useful in discriminating between low-risk or high-risk tumours and subsequent clinical outcomes.



Empire Genomics STAT6 Break Apart probe labeled in Spectrum Orange-Green was used in this publication.

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