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AuthorLuo, Wenyi
AuthorStevens, Todd M.
AuthorStafford, Phillip
AuthorMiettinen, Markku
AuthorGatalica, Zoran
AuthorVranic, Semir
Available date2021-11-29T11:17:51Z
Publication Date2021-12-01
Publication NameCurrent Oncology
Identifierhttp://dx.doi.org/10.3390/curroncol28060381
CitationLuo, W.; Stevens, T.M.; Stafford, P.; Miettinen, M.; Gatalica, Z.; Vranic, S. NUTM1-Rearranged Neoplasms: A Heterogeneous Group of Primitive Tumors with Expanding Spectrum of Histology and Molecular Alterations: An Updated Review. Curr. Oncol. 2021, 28, 4485–4503. https://doi.org/10.3390/ curroncol28060381
URIhttps://www.scopus.com/inward/record.uri?partnerID=HzOxMe3b&scp=85118900455&origin=inward
URIhttp://hdl.handle.net/10576/25192
AbstractNuclear protein of testis (NUT), a protein product of the NUTM1 gene (located on the long arm of chromosome 15) with highly restricted physiologic expression in post-meiotic spermatids, is the oncogenic driver of a group of emerging neoplasms when fused with genes involved in transcription regulation. Although initially identified in a group of lethal midline carcinomas in which NUT forms fusion proteins with bromodomain proteins, NUTM1-rearrangement has since been identified in tumors at non-midline locations, with non-bromodomain partners and with varied morphology. The histologic features of these tumors have also expanded to include sarcoma, skin adnexal tumors, and hematologic malignancies that harbor various fusion partners and are associated with markedly different clinical courses varying from benign to malignant. Most of these tumors have nondescript primitive morphology and therefore should be routinely considered in any undifferentiated neoplasm. The diagnosis is facilitated by the immunohistochemical use of the monoclonal C52 antibody, fluorescence in situ hybridization (FISH), and, recently, RNA-sequencing. The pathogenesis is believed to be altered expression of oncogenes or tumor suppressor genes by NUT-mediated genome-wide histone modification. NUTM1-rearranged neoplasms respond poorly to classical chemotherapy and radiation therapy. Targeted therapies such as bromodomain and extraterminal domain inhibitor (BETi) therapy are being developed. This current review provides an update on NUTM1-rearranged neoplasms, focusing on the correlation between basic sciences and clinical aspects.
Languageen
PublisherMDPI
SubjectNeoplasms
NUT protein
NUTM1 gene
Pathogenesis
Therapy
TitleNutm1-rearranged neoplasms: A heterogeneous group of primitive tumors with expanding spectrum of histology and molecular alterations: An updated review
TypeArticle
Issue Number6
Volume Number28
dc.accessType Open Access


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