Maccio Livia, Arciuolo Damiano, Santoro Angela, Raffone Antonio, Raimondo Diego, Ronchi Susanna, D'Alessandris Nicoletta, Scaglione Giulia, Valente Michele, Urtueta Belen Padial, Addante Francesca, Narducci Nadine, Bragantini Emma, Casarin Jvan, Angelico Giuseppe, La Rosa Stefano, Zannoni Gian Franco, Travaglino Antonio
Surgical Pathology Unit, S. Chiara Hospital, 38122 Trient, Italy.
Unità Operativa Complessa Anatomia Patologica Generale, Dipartimento di Scienze Della Salute Della Donna, del Bambino e di Sanità Pubblica, Fondazione Policlinico Universitario A. Gemelli IRCCS, 00168 Rome, Italy.
Diagnostics (Basel). 2025 Mar 20;15(6):792. doi: 10.3390/diagnostics15060792.
Among uterine tumors resembling ovarian sex cord tumors (UTROSCTs), it has been suggested that -rearranged cases are biologically distinct from -rearranged cases and might be considered as a separate entity. The aim of this systematic review was to assess the difference between - and -rearranged UTROSCTs with regard to several clinico-pathological parameters. Three electronic databases were searched from their inception to February 2025 for all studies assessing the presence of and rearrangements in UTROSCTs. Exclusion criteria comprised overlapping patient data, case reports, and reviews. Statistical analysis was performed to compare clinicopathological variables between - and -rearranged UTROSCTs. Dichotomous variables were compared by using Fisher's exact test; continuous variables were compared by using Student's -test. A -value < 0.05 was considered significant. Six studies with 88 molecularly classified UTROSCTs were included. A total of 36 cases were -rearranged, and 52 cases were -rearranged. -rearranged UTROSCTs showed a significantly older age ( < 0.001), larger tumor size ( = 0.002), less common submucosal/polypoid growth ( = 0.005), higher mitotic index ( = 0.010), more common LVSI ( = 0.049), and higher likelihood to undergo hysterectomy ( = 0.008) compared to -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). -rearranged UTROSCTs showed significantly lower disease-free survival compared to -rearranged UTROSTCs ( = 0.049). In conclusion, -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 -rearranged UTROSCTs. Nonetheless, the similar immunophenotype suggests that they belong to the same tumor family. Further studies are necessary to confirm this point.
在类似卵巢性索肿瘤的子宫肿瘤（UTROSCTs）中，有人提出，[此处原文可能缺失具体基因重排相关内容]重排的病例在生物学上与[此处原文可能缺失具体基因重排相关内容]重排的病例不同，可能应被视为一个独立的实体。本系统评价的目的是评估[此处原文可能缺失具体基因重排相关内容]重排和[此处原文可能缺失具体基因重排相关内容]重排的UTROSCTs在几个临床病理参数方面的差异。从数据库建立至2025年2月，对三个电子数据库进行检索，以查找所有评估UTROSCTs中[此处原文可能缺失具体基因重排相关内容]和[此处原文可能缺失具体基因重排相关内容]重排情况的研究。排除标准包括重叠的患者数据、病例报告和综述。进行统计分析以比较[此处原文可能缺失具体基因重排相关内容]重排和[此处原文可能缺失具体基因重排相关内容]重排的UTROSCTs之间的临床病理变量。二分变量采用Fisher精确检验进行比较；连续变量采用Student's t检验进行比较。P值<0.05被认为具有统计学意义。纳入了6项研究，共88例分子分类的UTROSCTs。其中36例为[此处原文可能缺失具体基因重排相关内容]重排，52例为[此处原文可能缺失具体基因重排相关内容]重排。与[此处原文可能缺失具体基因重排相关内容]重排的病例相比，[此处原文可能缺失具体基因重排相关内容]重排的UTROSCTs患者年龄显著更大（P<0.001），肿瘤尺寸更大（P = 0.002），黏膜下/息肉样生长较少见（P = 0.005），有丝分裂指数更高（P = 0.010），淋巴血管间隙浸润更常见（P = 0.049），接受子宫切除术的可能性更高（P = 0.008）。在切缘、细胞异型性、坏死、网状模式和横纹肌样细胞方面未检测到显著差异。在所评估的任何标志物（广谱细胞角蛋白、α-抑制素、钙视网膜蛋白、WT1、CD10、CD56、CD99、平滑肌肌动蛋白、结蛋白、h-钙调蛋白、Melan-A/MART1、SF1或Ki67）的免疫组化表达方面未发现显著差异。与[此处原文可能缺失具体基因重排相关内容]重排的UTROSTCs相比，[此处原文可能缺失具体基因重排相关内容]重排的UTROSCTs无病生存率显著更低（P = 0.049）。总之，与[此处原文可能缺失具体基因重排相关内容]重排的UTROSCTs相比，[此处原文可能缺失具体基因重排相关内容]重排的UTROSCTs发病年龄更大，黏膜下/息肉样生长的可能性更小，且肿瘤尺寸更大、有丝分裂指数更高、淋巴血管间隙浸润更常见、无病生存率更低。尽管如此，相似的免疫表型表明它们属于同一肿瘤家族。需要进一步研究来证实这一点。
