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

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

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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 breakapart 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 breakapart 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.

Original languageEnglish (US)
JournalJournal of Clinical Pathology
DOIs
StateAccepted/In press - Oct 31 2016

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ASJC Scopus subject areas

  • Pathology and Forensic Medicine

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Kouba, E., Simper, N. B., Chen, S., Williamson, S. R., Grignon, D., Eble, J., MacLennan, G. T., Montironi, R., Lopez-Beltran, A., Osunkoya, A. O., Zhang, S., Wang, M., Wang, L., Tran, T., Emerson, R., Baldrige, L. A., Monn, M. F., Linos, K., & Cheng, L. (Accepted/In press). Solitary fibrous tumour of the genitourinary tract: A clinicopathological study of 11 cases and their association with the NAB2-STAT6 fusion gene. Journal of Clinical Pathology. https://doi.org/10.1136/jclinpath-2016-204088