NAB2-STAT6 gene fusion in solitary fibrous tumors

A fusion between two particular genes occurs in at least half of all solitary fibrous tumors, according to two papers published this week in Nature Genetics. Solitary fibrous tumors (SFTs) are rare tumors that most often occur in the pleura, the membrane that covers the lung, but can also occur in any soft tissue site.

Arul Chinnaiyan and colleagues sequenced the exome of an SFT from a patient with recurrent tumors and detected a fusion between the NAB2 and STAT6 genes. Further analysis of a total of 51 SFTs identified a NAB2-STAT6 fusion in all cases. Additional experiments showed that expression of the NAB2-STAT6 fusion led to an increase in cell proliferation, providing further evidence that this fusion may be involved in the development of SFT.

Another study from Matthew Meyerson and colleagues reports whole-exome sequencing of 17 SFTs. Analysis of 53 tumors showed that the NAB2-STAT6 fusion was present in 29 (55%) of tumors.