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(American Journal of Pathology. 1998;153:1451-1458.)
© 1998 American Society for Investigative Pathology

Regular Articles

Congenital Mesoblastic Nephroma t(12;15) Is Associated with ETV6-NTRK3 Gene Fusion

Cytogenetic and Molecular Relationship to Congenital (Infantile)Fibrosarcoma

Brian P. Rubin*{dagger} , Chang-Jie Chen*{dagger} , Thomas W. Morgan* , Sheng Xiao*{dagger} , Holcombe E. Grier{ddagger}§ , Harry P. Kozakewich{ddagger}{dagger}, Antonio R. Perez-Atayde{ddagger}{dagger} and Jonathan A. Fletcher*{dagger}{ddagger}§

From the Departments of Pathology, Brigham and Women's Hospital* and Children's Hospital, and the Division of Pediatric Oncology,{ddagger} Dana-Farber Cancer Institute and Children's Hospital, and the Departments of Pathology{dagger} and Pediatrics,§ Harvard Medical School, Boston, Massachusetts

Morphological, cytogenetic, and biological evidence supports a relationship between congenital (infantile) fibrosarcoma (CFS) and congenital mesoblastic nephroma (CMN). These tumors have a very similar histological appearance, and they are both associated with polysomies for chromosomes 8, 11, 17, and 20. Recently, CFS was shown to contain a novel t(12;15)(p13;q25) translocation resulting in ETV6-NTRK3 gene fusion. The aims of this study were to determine whether congenital mesoblastic nephroma contains the t(12;15)(p13;q25) translocation and ETV6-NTRK3 gene fusion and whether ETV6-NTRK3 fusions, in CMN and CFS, antedate acquisition of nonrandom chromosome polysomies. To address these aims, we evaluated 1) ETV6-NTRK3 fusion transcripts by reverse transcriptase polymerase chain reaction and sequence analysis, 2) genomic ETV6-region chromosomal rearrangement by fluorescence in situ hybridization, and 3) chromosomal polysomies by karyotyping and fluorescence in situ hybridization. We report ETV6-NTRK3 fusion transcripts and/or ETV6-region rearrangement in five of six CMNs and in five of five CFSs. The ETV6-NTRK3 fusion transcripts and/or ETV-region chromosome rearrangements were demonstrated in two CMNs and one CFS that lacked chromosome polysomies. These findings demonstrate that t(12;15) translocation, and the associated ETV6-NTRK3 fusion, can antedate acquisition of chromosome polysomies in CMN and CFS. CMN and CFS are pathogenetically related, and it is likely that they represent a single neoplastic entity, arising in either renal or soft tissue locations.




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