• Yaoyao Fang, Xiaoli Zhou, Jing Bai, Qiuju Lin, Yingchun Zheng, Sijia Bo, Lei Sui, Ting Zhu, Nuo Bai, and Li Sun.
• School of Clinical Medicine, Shandong Second Medical University, Weifang, China.
• Medicine (Baltimore). 2024 Aug 9; 103 (32): e39093e39093.

RationaleEndometrial stromal sarcoma is an extremely rare mesenchymal neoplasm occurring in the extrauterine. Retroperitoneal endometrial stromal sarcoma with multiple pulmonary metastases, in particular, is extremely rare.Patient ConcernsForty-seven-year-old woman (gravida 3, para 2), was referred to our institution with complaints of fever.DiagnosesUltrasound and computed tomographic imaging of the abdomen identified the presence of masses in the pelvic region. Additionally, computed tomographic scans and X-ray evaluations of the thorax detected dispersed masses in both the lungs and pelvic area. Histopathological analysis of the pelvic region indicated the presence of low-grade endometrial stromal sarcoma. A computed tomography-guided pneumocentesis was conducted to further characterize the pulmonary lesions, confirming the diagnosis of low-grade endometrial stromal sarcoma.InterventionsThe patient underwent tumor resection, subsequent treatment with Medroxyprogesterone acetate for 6 months, received microwave ablation for multiple lung metastases, PARP1 inhibitor for 4 courses, and has been undergoing chemotherapy (epirubicin/ifosfamide) up to the present time.OutcomesPartial remission was obtained after the above treatment and this patient is now still alive maintaining for 3 years.LessonsThe identification and management of this disease remain a significant challenge due to its low prevalence. Further research involving a larger number of cases is necessary to ensure consistency in diagnosis and to establish effective treatment guidelines.Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.

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