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Genetic variant of SRF-rearranged myofibroma with a misleading nuclear expression of STAT6 and STAT6 involvement as 3' fusion partner.
Virchows Arch. 2020 Jun 11 [Online ahead of print]VA

Abstract

Pediatric neoplasms with a myofibroblastic differentiation are frequent in children, in particular myofibroma. Recently, a novel deep soft tissue myofibroblastic neoplasm has been described with high cellularity, a smooth muscle phenotype and SRF-RELA fusion. We report the case of a 15-year-old boy who presented with a tumor of the deep soft tissue of the arm, with overlapping histological features with the recently described SRF-RELA group of myofibromas but differing by the presence of calcifications, a novel SRF-STAT6 fusion transcript and nuclear expression of STAT6. No local recurrence nor distant metastasis was detected at the current follow-up of 29 months. The clinical relevance of this novel fusion requires further investigations.

Authors+Show Affiliations

Department of Pathology, INSERM, MMG, APHM, CHU Timone, Aix Marseille University, Marseille, France.Department of Pathology, INSERM, MMG, APHM, CHU Timone, Aix Marseille University, Marseille, France.Department of Pathology, Institut Bergonié, Bordeaux, France.Department of Pathology, INSERM, MMG, APHM, CHU Timone, Aix Marseille University, Marseille, France.Department of Pediatric orthopedic, APHM, La Timone Children's Hospital, Marseille, France.Department of Pediatric Hematology and Oncology, APHM, La Timone Children's Hospital, Marseille, France.Department of Radiology, APHM, Hopital Nord, Marseille, France.Department of Pathology, Institut Bergonié, Bordeaux, France.Department of Pathology, INSERM, MMG, APHM, CHU Timone, Aix Marseille University, Marseille, France. corinne.Bouvier2@ap-hm.fr. Service d'Anatomie & Cytologie Pathologiques, Neuropathologie, CHU Timone, Rue Saint-Pierre, 13005, Marseille, France. corinne.Bouvier2@ap-hm.fr.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

32529351

Citation

Nihous, Hugo, et al. "Genetic Variant of SRF-rearranged Myofibroma With a Misleading Nuclear Expression of STAT6 and STAT6 Involvement as 3' Fusion Partner." Virchows Archiv : an International Journal of Pathology, 2020.
Nihous H, Macagno N, Baud-Massière J, et al. Genetic variant of SRF-rearranged myofibroma with a misleading nuclear expression of STAT6 and STAT6 involvement as 3' fusion partner. Virchows Arch. 2020.
Nihous, H., Macagno, N., Baud-Massière, J., Haffner, A., Jouve, J. L., Gentet, J. C., Touzery, C., Le Loarer, F., & Bouvier, C. (2020). Genetic variant of SRF-rearranged myofibroma with a misleading nuclear expression of STAT6 and STAT6 involvement as 3' fusion partner. Virchows Archiv : an International Journal of Pathology. https://doi.org/10.1007/s00428-020-02859-9
Nihous H, et al. Genetic Variant of SRF-rearranged Myofibroma With a Misleading Nuclear Expression of STAT6 and STAT6 Involvement as 3' Fusion Partner. Virchows Arch. 2020 Jun 11; PubMed PMID: 32529351.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Genetic variant of SRF-rearranged myofibroma with a misleading nuclear expression of STAT6 and STAT6 involvement as 3' fusion partner. AU - Nihous,Hugo, AU - Macagno,Nicolas, AU - Baud-Massière,Jessica, AU - Haffner,Aurélie, AU - Jouve,Jean-Luc, AU - Gentet,Jean-Claude, AU - Touzery,Camille, AU - Le Loarer,François, AU - Bouvier,Corinne, Y1 - 2020/06/11/ PY - 2019/12/27/received PY - 2020/05/28/accepted PY - 2020/05/04/revised PY - 2020/6/13/entrez KW - Leiomyoma KW - Myofibroblastic proliferation KW - Myofibroma KW - Myopericytoma KW - Pediatric KW - RELA KW - SRF KW - STAT6 KW - Smooth muscle neoplasm JF - Virchows Archiv : an international journal of pathology JO - Virchows Arch. N2 - Pediatric neoplasms with a myofibroblastic differentiation are frequent in children, in particular myofibroma. Recently, a novel deep soft tissue myofibroblastic neoplasm has been described with high cellularity, a smooth muscle phenotype and SRF-RELA fusion. We report the case of a 15-year-old boy who presented with a tumor of the deep soft tissue of the arm, with overlapping histological features with the recently described SRF-RELA group of myofibromas but differing by the presence of calcifications, a novel SRF-STAT6 fusion transcript and nuclear expression of STAT6. No local recurrence nor distant metastasis was detected at the current follow-up of 29 months. The clinical relevance of this novel fusion requires further investigations. SN - 1432-2307 UR - https://www.unboundmedicine.com/medline/citation/32529351/Genetic_variant_of_SRF-rearranged_myofibroma_with_a_misleading_nuclear_expression_of_STAT6_and_STAT6_involvement_as_3'_fusion_partner L2 - https://dx.doi.org/10.1007/s00428-020-02859-9 DB - PRIME DP - Unbound Medicine ER -
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