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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
  1. Erik Kouba1,
  2. Novae B Simper1,
  3. Shaoxiong Chen1,
  4. Sean R Williamson3,
  5. David J Grignon1,
  6. John N Eble1,
  7. Gregory T MacLennan4,
  8. Rodolfo Montironi5,
  9. Antonio Lopez-Beltran6,7,
  10. Adeboye O Osunkoya8,
  11. Shaobo Zhang1,
  12. Mingsheng Wang1,
  13. Lisha Wang9,
  14. Thu Tran1,
  15. Robert E Emerson1,
  16. Lee Ann Baldrige1,
  17. M Francesca Monn2,
  18. Konstantinos Linos10,
  19. Liang Cheng1,2
  1. 1Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis, Indiana, USA
  2. 2Department of Urology, Indiana University School of Medicine, Indianapolis, Indiana, USA
  3. 3Department of Pathology and Laboratory Medicine, Henry Ford Health System, Detroit, Michigan, USA
  4. 4Departments of Pathology and Laboratory Medicine, Case Western Reserve University, Cleveland, Ohio, USA
  5. 5Department of Pathological Anatomy and Histopathology, School of Medicine, Polytechnic University of the Marche Region (Ancona), Ancona, Italy
  6. 6Unit of Anatomical Pathology, Department of Surgery, Faculty of Medicine, Cordoba, Spain
  7. 7Champalimaud Clinical Center, Lisbon, Portugal
  8. 8Department of Pathology, Emory University School of Medicine, Atlanta, Georgia, USA
  9. 9Michigan Center for Translational Pathology, University of Michigan, Ann Arbor, Michigan, USA
  10. 10Department of Pathology and Laboratory Medicine, Dartmouth-Hitchcock Medical Center, Geisel School of Medicine at Dartmouth, Hanover, New Hampshire, USA
  1. Correspondence to Dr Liang Cheng, Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, 350 West 11th Street, IUHPL Room 4010, Indianapolis, IN 46202, USA; liang_cheng{at}yahoo.com

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.

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Footnotes

  • Handling editor Cheok Soon Lee

  • Contributors All authors have contributed to the design and execution of the study.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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