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Non-canonical WNT6/WNT10A signal factor expression in EBV+ post-transplant smooth muscle tumors

Post-transplant smooth muscle tumors (PTSMTs) are rare mesenchymal neoplasms which occur after solid organ or haematopoietic stem cell transplantation. PTSMT typically consist of Epstein–Barr-virus (EBV)+ smooth muscle-like cells and show an intermediate malignancy. Their main occurrences are viscer...

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Detalles Bibliográficos
Autores principales: Teiken, Kristin, Kuehnel, Mark, Rehkaemper, Jan, Kreipe, Hans, Laenger, Florian, Hussein, Kais, Jonigk, Danny
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5985559/
https://www.ncbi.nlm.nih.gov/pubmed/29881541
http://dx.doi.org/10.1186/s13569-018-0096-8
Descripción
Sumario:Post-transplant smooth muscle tumors (PTSMTs) are rare mesenchymal neoplasms which occur after solid organ or haematopoietic stem cell transplantation. PTSMT typically consist of Epstein–Barr-virus (EBV)+ smooth muscle-like cells and show an intermediate malignancy. Their main occurrences are visceral organs, especially the liver, but intracranial appearances are described and associated with a poor prognosis. EBV drives the growth of PTSMT; however, the underlying molecular mechanisms still remain unclear. Gene expression analysis of a set of morphologically similar tumors (leiomyomas, leiomyosarcomas, angioleiomyomas and endothelial haemangiomas) from patients without immunosuppression or EBV-association was performed. Our findings indicate that PTSMT’s growth is driven by two factors of the wingless-type protein family: WNT6 and WNT10A. We are first to report that in PTSMTs, a non-canonical activation of WNT, independent of beta-catenin, drives tumor cell proliferation via MTOR/AKT1, MYC and Cyclin D2. ELECTRONIC SUPPLEMENTARY MATERIAL: The online version of this article (10.1186/s13569-018-0096-8) contains supplementary material, which is available to authorized users.