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卵巢微囊性间质瘤伴卵巢内复发及腹膜和大网膜播散:形态学、免疫组织化学和分子分析一例报告。

Ovarian Microcystic Stromal Tumor With Intraovarian Recurrence and Peritoneal and Omental Spread: A Case Report With Morphological, Immunohistochemical, and Molecular Analysis.

出版信息

Int J Gynecol Pathol. 2023 Sep 1;42(5):491-495. doi: 10.1097/PGP.0000000000000906. Epub 2022 Aug 9.

Abstract

Microcystic stromal tumors (MCSTs) are rare ovarian stromal tumors. They harbor CTNNB1 or APC mutations, resulting in β-catenin nuclear expression. To date, all MCST cases treated with oophorectomy or more extensive surgery have followed benign clinical courses. However, 1 of the 3 cases treated with ovarian cystectomy/tumor resection recurred in the residual ovary and iliac fossa 9 years after ovarian cystectomy. Here, we report a case of recurrent MCST in a 38-year-old woman. The patient underwent ovarian cystectomy for a 7.5 cm solid-cystic right ovarian mass, which showed classic morphological and immunophenotypical features of MCST. Four years later, the tumor recurred in the residual right ovary as a 21 cm mass, involving the pelvic peritoneum and omentum. Molecular analysis using next-generation sequencing revealed a single C TNNB1 exon 3 S37A mutation in the recurrent tumor. To the best of our knowledge, this is the second case of recurrent MCST, which presents more evidence that MCST has the potential to recur and spread locally. Rather than ovarian cystectomy/tumor resection, more aggressive surgery, such as unilateral oophorectomy, may be necessary to decrease the risk of recurrence. Long-term postsurgery follow up is needed, especially after simple ovarian cystectomy/tumor resection.

摘要

微囊型卵巢基质肿瘤(MCSTs)是一种罕见的卵巢基质肿瘤。它们携带 CTNNB1 或 APC 突变,导致 β-连环蛋白核表达。迄今为止,所有接受卵巢切除术或更广泛手术治疗的 MCST 病例均表现出良性的临床病程。然而,在接受卵巢囊肿切除术/肿瘤切除术治疗的 3 例病例中,有 1 例在卵巢囊肿切除术后 9 年在残留卵巢和髂窝复发。在此,我们报告 1 例 38 岁女性复发性 MCST 的病例。该患者因 7.5cm 实性-囊性右侧卵巢肿块而行卵巢囊肿切除术,该肿块表现出 MCST 的典型形态学和免疫表型特征。4 年后,肿瘤在残留的右侧卵巢中复发,大小为 21cm,累及盆腔腹膜和大网膜。使用下一代测序进行的分子分析显示复发性肿瘤中存在单个 C TNNB1 外显子 3 S37A 突变。据我们所知,这是第二例复发性 MCST 病例,进一步证明 MCST 有局部复发和播散的潜力。为降低复发风险,可能需要更积极的手术,如单侧卵巢切除术,而不是卵巢囊肿切除术/肿瘤切除术。需要进行长期的术后随访,尤其是在单纯进行卵巢囊肿切除术/肿瘤切除术之后。

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