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卵巢微囊性间质瘤伴网膜转移:首例病例报告及文献复习。

Ovarian microcystic stromal tumor with omental metastasis: the first case report and literature review.

机构信息

Department of Oncologic Gynecology, The First Hospital of Jilin University, Xinmin Street 1,Changchun, Jilin, 130021, People's Republic of China.

Department of Cardiac Surgery, The First Hospital of Jilin University, Changchun, 130021, Jilin, People's Republic of China.

出版信息

J Ovarian Res. 2021 May 27;14(1):73. doi: 10.1186/s13048-021-00812-1.

Abstract

BACKGROUND

Microcystic stromal tumor (MCST) of the ovary is an extremely rare subtype of sex cord-stromal neoplasm first described by Irving and Young in 2009. Tumors from all previously reported cases (fewer than 40 total) were benign, but one was a case of ovarian MCST that reoccurred.

CASE PRESENTATION

Herein, we present a unique single case of ovarian MCST with omental metastasis in a 47-year-old Chinese female along with its histologic and immunohistochemical profile and genetic alterations. The tumor exhibited the previously described classic microscopic features and immunoprofiles of MCST. The tumorlet in the omentum presented the same histological structures and characteristically expressed β-catenin protein (localized in the nucleus). Molecular analysis identified a point mutation (c.98C > G) in exon 3 of CTNNB1.

CONCLUSIONS

To the best of our knowledge, no such report has been documented for ovarian MCST with omental metastasis. The study may provide new insights into the tumor biology of MCST and provide a better understanding of this rare entity.

摘要

背景

卵巢微囊性间质肿瘤(MCST)是一种极其罕见的性索-间质肿瘤亚型,于 2009 年由 Irving 和 Young 首次描述。此前报道的所有肿瘤(总数少于 40 例)均为良性,但有一例卵巢 MCST 复发。

病例介绍

在此,我们报告了一例独特的卵巢 MCST 伴网膜转移的病例,患者为 47 岁中国女性,其组织学和免疫组化特征及基因改变如下。肿瘤表现出先前描述的 MCST 的经典微观特征和免疫表型。网膜内的肿瘤小结呈现出相同的组织学结构,并特征性地表达β-连环蛋白蛋白(定位于细胞核)。分子分析确定 CTNNB1 外显子 3 中有一个点突变(c.98C>G)。

结论

据我们所知,目前尚无卵巢 MCST 伴网膜转移的相关报道。该研究可能为 MCST 的肿瘤生物学提供新的见解,并更好地了解这一罕见实体。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1c77/8157402/58b8e32272c5/13048_2021_812_Fig1_HTML.jpg

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