Novel high-grade endometrial stromal sarcoma: a morphologic mimicker of myxoid leiomyosarcoma

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dc.contributor.authorHoang, Lien N.
dc.contributor.authorAneja, Amandeep
dc.contributor.authorConlon, Niamh
dc.contributor.authorDelair, Deborah F.
dc.contributor.authorMiddha, Sumit
dc.contributor.authorBenayed, Ryma
dc.contributor.authorHensley, Martee L.
dc.contributor.authorPark, Kay J.
dc.contributor.authorHollmann, Travis J.
dc.contributor.authorHameed, Meera R.
dc.contributor.authorAntonescu, Cristina R.
dc.contributor.authorSoslow, Robert A.
dc.contributor.authorChiang, Sarah
dc.date.accessioned2018-06-05T08:37:10Z
dc.date.available2018-06-05T08:37:10Z
dc.date.issued2017-01
dc.description.abstractEndometrial stromal sarcomas (ESS) are often underpinned by recurrent chromosomal translocations resulting in the fusion of genes involved in epigenetic regulation. To date, only YWHAE-NUTM2 rearrangements are associated with distinctive high-grade morphology and aggressive clinical behavior. We identified 3 ESS morphologically mimicking myxoid leiomyosarcoma of the uterus and sought to describe their unique histopathologic features and identify genetic alterations using next-generation sequencing. All cases displayed predominantly spindled cells associated with abundant myxoid stroma and brisk mitotic activity. Tumors involved the endometrium and demonstrated tongue-like myometrial infiltration. All 3 were associated with an aggressive clinical course, including multisite bony metastases in 1 patient, progressive peritoneal disease after chemotherapy in another and metastases to the lung and skin in the last patient. All 3 ESS were found to harbor ZC3H7B-BCOR gene fusions by targeted sequencing and fluorescence in situ hybridization. On the basis of the review of these cases, we find that ESS with ZC3H7B-BCOR fusion constitutes a novel type of high-grade ESS and shares significant morphologic overlap with myxoid leiomyosarcoma.en
dc.description.statusPeer revieweden
dc.description.versionAccepted Versionen
dc.format.mimetypeapplication/pdfen
dc.identifier.citationHoang, L. N., Aneja, A., Conlon, N., Delair, D. F., Middha, S., Benayed, R., Hensley, M. L., Park, K. J., Hollmann, T. J., Hameed, M. R., Antonescu, C. R., Soslow, R. A. and Chiang, S. (2017) ‘Novel high-grade endometrial stromal sarcoma: a morphologic mimicker of myxoid leiomyosarcoma’, American Journal of Surgical Pathology, 41(1), pp. 12-24. doi: 10.1097/PAS.0000000000000721en
dc.identifier.doi10.1097/PAS.0000000000000721
dc.identifier.endpage24en
dc.identifier.issn0147-5185
dc.identifier.issn1532-0979
dc.identifier.issued1en
dc.identifier.journaltitleAmerican Journal of Surgical Pathologyen
dc.identifier.startpage12en
dc.identifier.urihttps://hdl.handle.net/10468/6242
dc.identifier.volume41en
dc.language.isoenen
dc.publisherLippincott, Williams & Wilkinsen
dc.rights© 2017, Wolters Kluwer Health, Inc. All rights reserved.en
dc.subjectEndometrial stromal sarcomaen
dc.subjectUterine sarcomaen
dc.subjectZC3H7Ben
dc.subjectBCORen
dc.subjectTranslocationen
dc.subjectMyxoid leiomyosarcomaen
dc.subjectPolycomb repressor complexen
dc.titleNovel high-grade endometrial stromal sarcoma: a morphologic mimicker of myxoid leiomyosarcomaen
dc.typeArticle (peer-reviewed)en
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