Department of Gynaecology and Obstetrics, Sygehus Sønderjylland i Aabenraa, Aabenraa, Denmark.
Department of Clinical Genetics, Lillebaelt Hospital-University Hospital of Southern Denmark, Vejle, Denmark.
Int J Gynecol Cancer. 2023 Dec 4;33(12):1921-1927. doi: 10.1136/ijgc-2023-004945.
Sertoli-Leydig cell tumors are rare tumors of the ovary. Moderate and poorly differentiated tumors can metastasize and have a poor outcome. A pathogenic variant in is associated with an increased risk of developing these tumors along with other clinical phenotypes. We aimed to describe a national cohort of all Sertoli-Leydig cell tumors with regard to clinicopathological characteristics and frequency of pathogenic variants.
In May 2018, all patients registered from January 1997 to December 2017 with the Systematized Nomenclature of Medicine code M86310 (Sertoli-Leydig cell tumor) were obtained from the Danish National Pathology Registry. Validation of the diagnosis depended on comments in the reports that two pathologists validated the initial diagnosis or revision of the pathology at another facility. We performed descriptive statistics to describe baseline characteristics, and cancer related survival was calculated using Kaplan-Meier analysis followed by a log rank test for differences between variables RESULTS: 41 women with Sertoli-Leydig cell tumors were identified. Median age was 41 years (range 6-79). The stages according to the International Federation of Gynecology and Obstetrics (FIGO) were: stage I, 85% (n=35), stage II, 2% (n=1), stage III, 5% (n=2), and stage IV, 7% (n=3). The 5 year cancer related survival was 100% for patients with localized disease (stages I-II) and 0% in advanced tumor stages (stages III-IV). Histological differentiation grade of the tumors was well differentiated in 29% (n=12), moderately differentiated in 56% (n=23), and poorly differentiated in 15% (n=6), and the 5 year cancer related survival was 100%, 96%, and 33%, respectively, according to grade. All patients underwent surgery. Twenty-two patients had fertility sparing surgery and four of these had given birth at the time of follow-up. Analysis of was performed in eight women. Four carried a pathogenic variant. Four patients received adjuvant chemotherapy, three because of advanced tumor stage, and one because of a poorly differentiated Sertoli-Leydig cell tumor.
The prognosis for women with Sertoli-Leydig cell tumors with localized disease is excellent. Women with advanced stages (III-IV) have a poor prognosis, regardless of adjuvant chemotherapy. Fertility sparing surgery seems to be a viable option for localized Sertoli-Leydig cell tumors. screening was rarely performed in previous cohorts and concomitant organ screening programs are topics for discussion.
支持细胞-间质细胞瘤是卵巢罕见的肿瘤。中、低分化肿瘤可转移，预后不良。 种系变异与这些肿瘤的发生以及其他临床表型相关。我们旨在描述一个全国性的支持细胞-间质细胞瘤队列，包括临床病理特征和 种系变异的频率。
2018 年 5 月，从丹麦国家病理登记处获取了 1997 年 1 月至 2017 年 12 月期间使用医学系统命名法代码 M86310（支持细胞-间质细胞瘤）登记的所有患者。诊断的验证取决于报告中的注释，即两位病理学家验证了初始诊断或在另一家机构修改了病理学。我们进行了描述性统计，以描述基线特征，并使用 Kaplan-Meier 分析计算癌症相关生存率，然后使用对数秩检验比较变量之间的差异。结果：共发现 41 名支持细胞-间质细胞瘤患者。中位年龄为 41 岁（范围 6-79 岁）。国际妇产科联盟（FIGO）分期为：I 期 85%（n=35），II 期 2%（n=1），III 期 5%（n=2），IV 期 7%（n=3）。局部疾病（I-II 期）患者的 5 年癌症相关生存率为 100%，晚期肿瘤患者（III-IV 期）为 0%。肿瘤的组织学分化程度为高分化 29%（n=12），中分化 56%（n=23），低分化 15%（n=6），相应的 5 年癌症相关生存率为 100%、96%和 33%。所有患者均接受手术治疗。22 名患者行保留生育力手术，其中 4 名在随访时已分娩。对 8 名女性进行了 分析。4 名携带致病性变异。4 名患者接受辅助化疗，3 名患者因晚期肿瘤，1 名患者因低分化支持细胞-间质细胞瘤。结论：局限性疾病女性支持细胞-间质细胞瘤的预后良好。晚期（III-IV 期）患者预后不良，无论是否接受辅助化疗。保留生育力手术似乎是局限性支持细胞-间质细胞瘤的一种可行选择。 筛查在以前的队列中很少进行，同时进行器官筛查计划是讨论的主题。
