PDF(Pubmed)
Abstract：
BACKGROUND: Endometrial 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.
METHODS: Forty-seven-year-old woman (gravida 3, para 2), was referred to our institution with complaints of fever.
METHODS: Ultrasound 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.
METHODS: The 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.
RESULTS: Partial remission was obtained after the above treatment and this patient is now still alive maintaining for 3 years.
CONCLUSIONS: The 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.
METHODS: Forty-seven-year-old woman (gravida 3, para 2), was referred to our institution with complaints of fever.
METHODS: Ultrasound 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.
METHODS: The 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.
RESULTS: Partial remission was obtained after the above treatment and this patient is now still alive maintaining for 3 years.
CONCLUSIONS: The 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.
摘要：
背景：子宫内膜间质肉瘤是一种发生在子宫外的极为罕见的间充质肿瘤。腹膜后子宫内膜间质肉瘤伴多发肺转移,特别是,极为罕见。
方法：47岁妇女（gravida3，第2段），被转介到我们的机构，投诉发烧。
方法：腹部超声和计算机断层成像确定盆腔区域存在肿块。此外,胸部的计算机断层扫描和X线评估在肺部和骨盆区域发现了分散的肿块。盆腔区域的组织病理学分析表明存在低度子宫内膜间质肉瘤。进行计算机断层扫描引导的肺穿刺术以进一步表征肺部病变，确认低度子宫内膜间质肉瘤的诊断。
方法：患者接受肿瘤切除术，随后用醋酸甲羟孕酮治疗6个月，接受微波消融治疗多发性肺转移瘤，PARP1抑制剂4个疗程，一直在接受化疗（表柔比星/异环磷酰胺）。
结果：上述治疗后获得部分缓解，该患者现在仍然存活3年。
结论：由于该疾病的低患病率，其识别和管理仍然是一个重大挑战。需要进行涉及更多病例的进一步研究，以确保诊断的一致性并建立有效的治疗指南。
方法：47岁妇女（gravida3，第2段），被转介到我们的机构，投诉发烧。
方法：腹部超声和计算机断层成像确定盆腔区域存在肿块。此外,胸部的计算机断层扫描和X线评估在肺部和骨盆区域发现了分散的肿块。盆腔区域的组织病理学分析表明存在低度子宫内膜间质肉瘤。进行计算机断层扫描引导的肺穿刺术以进一步表征肺部病变，确认低度子宫内膜间质肉瘤的诊断。
方法：患者接受肿瘤切除术，随后用醋酸甲羟孕酮治疗6个月，接受微波消融治疗多发性肺转移瘤，PARP1抑制剂4个疗程，一直在接受化疗（表柔比星/异环磷酰胺）。
结果：上述治疗后获得部分缓解，该患者现在仍然存活3年。
结论：由于该疾病的低患病率，其识别和管理仍然是一个重大挑战。需要进行涉及更多病例的进一步研究，以确保诊断的一致性并建立有效的治疗指南。
