Int J Gynecol Pathol. 2023 Sep 1;42(5):500-507. doi: 10.1097/PGP.0000000000000910. Epub 2022 Aug 22.
We report a case of a cystic ovarian neoplasm in a 76-yr-old female composed of 2 distinct and intimately associated components: a macrocystic adult granulosa cell tumor (AGCT) and a serous borderline tumor. The granulosa cell nature of the tumor was confirmed with positive immunohistochemical staining for inhibin, calretinin, and WT1, while the neoplastic nature of the granulosa cell proliferation was supported by the presence of a point mutation of the FOXL2 gene. A review of 19 previously reported mixed AGCT and epithelial neoplasms of the ovary is included. Of the eight mixed AGCT and epithelial tumors, including our case, that were tested for FOXL2 mutation, 4 of the 5 mutation-positive cases were notable for demonstrating a macroscopically visible nodule or mass of AGCT at the time of gross examination, while 2 of the 3 mutation-negative cases lacked a mass-producing granulosa cell component. This feature by itself may be sufficient to predict the true neoplastic nature of the granulosa cell proliferation. This is the first reported case of a composite neoplastic AGCT and serous borderline tumor. We also discuss the current histogenetic models for these rare mixed AGCT and epithelial tumors.
我们报告了一例 76 岁女性的囊性卵巢肿瘤，由 2 个截然不同但密切相关的成分组成：一个大囊性腺泡细胞肿瘤（AGCT）和一个浆液性交界性肿瘤。肿瘤的颗粒细胞性质通过抑制素、钙视网膜蛋白和 WT1 的阳性免疫组织化学染色得到证实，而颗粒细胞增殖的肿瘤性质则得到 FOXL2 基因突变的存在支持。还包括对 19 例先前报道的混合 AGCT 和卵巢上皮性肿瘤的回顾。在对 FOXL2 突变进行检测的 8 例混合 AGCT 和上皮性肿瘤中，包括我们的病例，在大体检查时，有 4/5 的突变阳性病例有肉眼可见的 AGCT 结节或肿块，而 3/3 的突变阴性病例缺乏产生颗粒细胞的成分。这一特征本身可能足以预测颗粒细胞增殖的真正肿瘤性质。这是首例报道的复合性 AGCT 和浆液性交界性肿瘤。我们还讨论了这些罕见的混合 AGCT 和上皮性肿瘤的当前组织发生模型。
