Clinicopathological Comparison Between <i>GREB1</i>- and <i>ESR1</i>-Rearranged Uterine Tumors Resembling Ovarian Sex Cord Tumors (UTROSCTs): A Systematic Review

Clinicopathological Comparison Between <i>GREB1</i>- and <i>ESR1</i>-Rearranged Uterine Tumors Resembling Ovarian Sex Cord Tumors (UTROSCTs): A Systematic Review

<b>Introduction:</b> Among uterine tumors resembling ovarian sex cord tumors (UTROSCTs), it has been suggested that <i>GREB1</i>-rearranged cases are biologically distinct from <i>ESR1</i>-rearranged cases and might be considered as a separate entity. <b>Obj...

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Main Authors: Livia Maccio, Damiano Arciuolo, Angela Santoro, Antonio Raffone, Diego Raimondo, Susanna Ronchi, Nicoletta D’Alessandris, Giulia Scaglione, Michele Valente, Belen Padial Urtueta, Francesca Addante, Nadine Narducci, Emma Bragantini, Jvan Casarin, Giuseppe Angelico, Stefano La Rosa, Gian Franco Zannoni, Antonio Travaglino
Format: Article
Language:English
Published: MDPI AG 2025-03-01
Series:Diagnostics
Subjects:
UTROSCTs
<i>GREB1</i>
<i>ESR1</i>
clinicopathological
immunohistochemistry
Online Access:https://www.mdpi.com/2075-4418/15/6/792
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Summary:<b>Introduction:</b> Among uterine tumors resembling ovarian sex cord tumors (UTROSCTs), it has been suggested that <i>GREB1</i>-rearranged cases are biologically distinct from <i>ESR1</i>-rearranged cases and might be considered as a separate entity. <b>Objectives:</b> The aim of this systematic review was to assess the difference between <i>GREB1</i>- and <i>ESR1</i>-rearranged UTROSCTs with regard to several clinico-pathological parameters. <b>Methods:</b> Three electronic databases were searched from their inception to February 2025 for all studies assessing the presence of <i>GREB1</i> and <i>ESR1</i> rearrangements in UTROSCTs. Exclusion criteria comprised overlapping patient data, case reports, and reviews. Statistical analysis was performed to compare clinicopathological variables between <i>GREB1</i>- and <i>ESR1</i>-rearranged UTROSCTs. Dichotomous variables were compared by using Fisher’s exact test; continuous variables were compared by using Student’s <i>t</i>-test. A <i>p</i>-value < 0.05 was considered significant. <b>Results:</b> Six studies with 88 molecularly classified UTROSCTs were included. A total of 36 cases were <i>GREB1</i>-rearranged, and 52 cases were <i>ESR1</i>-rearranged. <i>GREB1</i>-rearranged UTROSCTs showed a significantly older age (<i>p</i> < 0.001), larger tumor size (<i>p</i> = 0.002), less common submucosal/polypoid growth (<i>p</i> = 0.005), higher mitotic index (<i>p</i> = 0.010), more common LVSI (<i>p</i> = 0.049), and higher likelihood to undergo hysterectomy (<i>p</i> = 0.008) compared to <i>ESR1</i>-rearranged cases. No significant differences were detected with regard to margins, cytological atypia, necrosis, retiform pattern, and rhabdoid cells. No significant differences were found in the immunohistochemical expression of any of the assessed markers (wide-spectrum cytokeratins, α-inhibin, calretinin, WT1, CD10, CD56, CD99, smooth muscle actin, desmin, h-caldesmon, Melan-A/MART1, SF1, or Ki67). <i>GREB1</i>-rearranged UTROSCTs showed significantly lower disease-free survival compared to <i>ESR1</i>-rearranged UTROSTCs (<i>p</i> = 0.049). <b>Conclusions:</b> In conclusion, <i>GREB1</i>-rearranged UTROSCTs occur at an older age, are less likely to display a submucosal/polypoid growth, and exhibit larger size, a higher mitotic index, more common lymphovascular space invasion, and lower disease-free survival compared to <i>ESR1</i>-rearranged UTROSCTs. Nonetheless, the similar immunophenotype suggests that they belong to the same tumor family. Further studies are necessary to confirm this point.
ISSN:2075-4418

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