Solitary fibrous tumour of the genitourinary tract: a clinicopathological study of 11 cases and their association with the NAB2-STAT6 fusion gene. Issue 6 (31st October 2016)

• Record Type: Journal Article
• Authors: Kouba, Erik; Simper, Novae B; Chen, Shaoxiong; Williamson, Sean R; Grignon, David J; Eble, John N; MacLennan, Gregory T; Montironi, Rodolfo; Lopez-Beltran, Antonio; Osunkoya, Adeboye O; Zhang, Shaobo; Wang, Mingsheng; Wang, Lisha; Tran, Thu; Emerson, Robert E; Baldrige, Lee Ann; Monn, M Francesca; Linos, Konstantinos; Cheng, Liang
• Is Part Of: Journal of clinical pathology. Volume 70:Issue 6(2017)
• Page Start: 508
• Page End: 514
• Publication Date: 2016-10-31
• Subjects: KIDNEY -- CYTOGENETICS -- IN SITU HYBRIDISATION -- GENITOURINARY PATHOLOGY; Pathology -- Periodicals; Pathology, Molecular -- Periodicals; 616.0705
• DOI: 10.1136/jclinpath-2016-204088
• Languages: English
• ISSNs: 0021-9746

Abstract

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.