CASE REPORT article
Front. Oncol.
Sec. Gynecological Oncology
A Rare Case of Bilateral Primary Müllerian adenosarcoma of the ovary with Uncommon Extensive Metastasis and GNAQ Mutation
Provisionally accepted
- Renmin Hospital of Wuhan University, Wuhan, China
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Word count: 260 Background: Müllerian adenosarcoma (MA) is a rare low-grade malignant tumor characterized by benign glandular epithelium and malignant sarcomatous stromal components, commonly arising in the uterine corpus. Primary ovarian MA is uncommon and typically presents as a unilateral lesion (FIGO stages I-III). However, bilateral ovarian involvement coupled with extensive metastasis in ovarian adenosarcoma has not been previously reported.Case presentation: This article reported a rare case of bilateral primary ovarian adenosarcoma with extensive metastasis, accompanied by lymphovascular invasion (LVI). Histopathological examination revealed a tumor composed of proliferative glands and stroma. The glandular epithelium was separated by abundant stromal components, forming a lobulated architecture resembling breast phyllodes tumor. The glandular epithelial cells exhibited a single-layered columnar morphology with bland cytological features. The stromal cells displayed a spindle-shaped morphology, arranged densely around spiral artery-like vessels, with mild atypia and visible mitotic figures. Notably, periglandular stromal condensation formed a characteristic "cuff-like" pattern. Extensive metastases were identified in the subumbilical region, intestinal tract, omentum, and distal segment of the right ureter. Significantly, tumor emboli were detected within vascular channels beneath the rectal serosa. Subsequently, next-generation sequencing (NGS) analysis revealed a novel mutation in the GNAQ (previously unreported in this tumor type). Additionally, immunohistochemical (IHC) analysis confirmed positive PD-L1 expression. Finally, the patient received a comprehensive treatment regimen comprising radiotherapy, chemotherapy and immunotherapy.We describe a unique case of ovarian adenosarcoma presenting with bilateral ovarian involvement, manifesting at the more advanced stage IIIC, notably in the absence of sarcomatous overgrowth (SO). Moreover, we report for the first time the presence of LVI and the GNAQ missense variant in ovarian adenosarcoma.
Keywords: Ovary, Adenosarcoma, metastasis, lymphovascular invasion, Mutation
Received: 02 Jul 2025; Accepted: 17 Nov 2025.
Copyright: © 2025 Ai, Li, He, Yuan, Yan and Zhang. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence: Xiuyun Zhang, 807625410@qq.com
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