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卵巢性索-间质肿瘤：支持细胞-间质细胞瘤

Author
David M Gershenson, MD
Section Editors
Barbara Goff, MD
Rochelle L Garcia, MD
Deputy Editor
Sandy J Falk, MD, FACOG
Translators
赵恩锋, 主任医师，教授

引言

卵巢性索-间质肿瘤是一组具有异质性的良性或恶性肿瘤，来源于干细胞(通常产生包围卵母细胞的细胞)，包括产生卵巢激素的细胞(性腺的非生殖细胞和非上皮成分) (图 1)[1]。卵巢性索-间质肿瘤实属罕见，仅占所有原发性卵巢癌的1.2%[2]。

与上皮性卵巢癌不同，大多数恶性卵巢性索-间质肿瘤患者在诊断时尚处于早期，并且通常认为这些肿瘤为低级别恶性肿瘤。

支持细胞-间质细胞瘤占卵巢肿瘤的比例不到0.5%[3]。这些肿瘤可能表现为良性或恶性，这与个体病例中肿瘤的分化程度有关。近75%的肿瘤发生于40岁以下的女性患者(平均诊断年龄为25岁)，但是在各年龄段均有发病。这种肿瘤以存在产生雄激素的睾丸结构为特征。这可导致男性化，但并非所有的肿瘤都可活跃分泌激素。

支持细胞-间质细胞类型的卵巢性索-间质肿瘤将总结在此。这类肿瘤还包括支持细胞-间质细胞瘤(卵巢男性母细胞瘤)和单纯的支持细胞瘤或者间质细胞瘤。性索-间质肿瘤的概述、其他类型的卵巢性索-间质肿瘤(支持细胞-间质细胞瘤、颗粒细胞瘤与支持细胞-间质细胞瘤混合型)以及上皮性卵巢癌将单独讨论。 (参见“卵巢性索-间质肿瘤概述”和“卵巢性索-间质肿瘤：颗粒细胞-间质细胞肿瘤”和“卵巢性索-间质肿瘤：颗粒细胞及支持-间质细胞混合型肿瘤”和“上皮性卵巢、输卵管和腹膜癌的组织病理学”和“上皮性卵巢癌、输卵管癌及腹膜癌的临床特征和诊断”)

遗传学

据报道，有两种基因突变与支持细胞-间质细胞瘤有关，且两种突变可能均具有临床相关性。FOXL2是最初被报道存在于卵巢颗粒细胞瘤中的一种体细胞性错义点突变[4]。随后，同样的研究组在一项更大型的卵巢性索-间质肿瘤的病例系列研究中分析了该标记物[5]。作者们发现FOXL2是一种对此类肿瘤具有相对敏感性且高度特异性的标记物。FOXL2免疫染色存在于几乎所有伴FOXL2基因突变的性索-间质肿瘤和大部分无突变肿瘤。重要的是，仅50%的支持细胞-间质细胞瘤表达了FOXL2基因。然而，FOXL2与α-抑制素和钙视网膜蛋白的联合将成为有助于诊断卵巢性索-间质肿瘤的免疫标记物。

          

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Literature review current through: 2017-06 . | This topic last updated: 2017-02-07.
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