目的:低级别和高级别子宫内膜间质肉瘤(LGESS和HGESS)和未分化子宫肉瘤(UUS)是近年来病理分类和分期系统发生改变的罕见肿瘤。据报道这些肿瘤含有融合基因。我们的目的是澄清遗传背景,临床特征,预后因素,以及使用新的分类和分期系统对这些肿瘤进行最佳治疗。
方法:我们分析了72例LGESS患者的临床特征和预后信息,25与HGESS,和16使用中央病理检查的UUS。通过免疫组织化学检查雌激素和孕激素受体(PgRs)。使用实时聚合酶链反应测试JAZF1-SUZ12和YWHAE-NUTM2A/B基因融合。
结果:LGESS的5年总生存率,HGESS,UUS为94%,53%,25%,分别。在LGESS,第四阶段,不完整的手术,PgR缺失与不良OS相关。JAZF1-SUZ12融合基因的存在与OS无关。在HGESS,分期与预后的关系尚不清楚.3例YWHAE-NUTM2A/B融合基因患者随访期间均无死亡。辅助化疗与良好的OS相关。UUS的不完全切除与不良OS相关;然而,经常发生残留肿瘤。尽管大多数患者接受了辅助化疗,即使在I期疾病中,其预后也极差.
结论:LGESS的预后通常良好;然而,第四阶段,不完整的手术,PgR阴性肿瘤与不良预后相关。辅助化疗可能对HGESS有用。UUS的预后极差,即使是辅助化疗.
Low-grade and high-grade endometrial stromal sarcomas (LGESS and HGESS) and undifferentiated uterine sarcomas (UUS) are rare tumors whose pathological classification and staging system have changed recently. These tumors are reported to contain fusion genes. We aimed to clarify the genetic background, clinical features, prognostic factors, and optimal therapy of these tumors using a new classification and staging system.
We analyzed the clinical features and prognostic information of 72 patients with LGESS, 25 with HGESS, and 16 with UUS using central pathological
review. Estrogen and progesterone receptors (PgRs) were examined by immunohistochemistry. JAZF1-SUZ12 and YWHAE-NUTM2A/B gene fusions were tested using real-time polymerase chain reaction.
The 5-year overall survival (OS) rates of LGESS, HGESS, and UUS were 94%, 53%, and 25%, respectively. In LGESS, stage IV, incomplete surgery, and absence of PgR were associated with poor OS. The presence of JAZF1-SUZ12 fusion gene was not associated with OS. In HGESS, the relationship between stage and prognosis was unclear. None of the 3 patients with YWHAE-NUTM2A/B fusion gene died during follow-up. Adjuvant chemotherapy was associated with a favorable OS. Incomplete resection of UUS was associated with poor OS; however, residual tumors frequently occurred. Although most patients underwent adjuvant chemotherapy, their prognosis was extremely poor even in stage I disease.
Prognosis of LGESS is generally good; however, stage IV, incomplete surgery, and PgR-negative tumors are associated with poor prognosis. Adjuvant chemotherapy may be useful for HGESS. Prognosis of UUS is extremely poor, even with adjuvant chemotherapy.