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

Show simple item record Hoang, Lien N. Aneja, Amandeep Conlon, Niamh Delair, Deborah F. Middha, Sumit Benayed, Ryma Hensley, Martee L. Park, Kay J. Hollmann, Travis J. Hameed, Meera R. Antonescu, Cristina R. Soslow, Robert A. Chiang, Sarah 2018-06-05T08:37:10Z 2018-06-05T08:37:10Z 2017-01
dc.identifier.citation Hoang, 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.0000000000000721 en
dc.identifier.volume 41 en
dc.identifier.issued 1 en
dc.identifier.startpage 12 en
dc.identifier.endpage 24 en
dc.identifier.issn 0147-5185
dc.identifier.issn 1532-0979
dc.identifier.doi 10.1097/PAS.0000000000000721
dc.description.abstract Endometrial 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.format.mimetype application/pdf en
dc.language.iso en en
dc.publisher Lippincott, Williams & Wilkins en
dc.rights © 2017, Wolters Kluwer Health, Inc. All rights reserved. en
dc.subject Endometrial stromal sarcoma en
dc.subject Uterine sarcoma en
dc.subject ZC3H7B en
dc.subject BCOR en
dc.subject Translocation en
dc.subject Myxoid leiomyosarcoma en
dc.subject Polycomb repressor complex en
dc.title Novel high-grade endometrial stromal sarcoma: a morphologic mimicker of myxoid leiomyosarcoma en
dc.type Article (peer-reviewed) en
dc.internal.authorcontactother Niamh Conlon, Pathology, University College Cork, Cork, Ireland. T: +353-21-490-3000 en
dc.internal.availability Full text available en
dc.description.version Accepted Version en
dc.description.status Peer reviewed en
dc.identifier.journaltitle American Journal of Surgical Pathology en

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