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病例报告:静脉内平滑肌瘤病从子宫延伸至右心房。

A case report: intravenous leiomyomatosis extending from the uterus to the right atrium.

作者信息

Ziani Hicham, El Idrissi Jallal Nora, Lahbabi Yassir, Slaihi Zakariae, Lahbabi Sophia, Oudghiri Nezha, Tachinante Rajae

机构信息

Department of Intensive Care Unit of Maternity, CHU IBN SINA, Mohammed V Souissi University, Rabat, Morocco.

出版信息

Ann Med Surg (Lond). 2024 Feb 5;86(3):1766-1770. doi: 10.1097/MS9.0000000000001783. eCollection 2024 Mar.

Abstract

INTRODUCTION AND IMPORTANCE

Intra veinous leiomyomatosis (IVL) is one pathology of a known group of ectopic leiomyomatosis that gathers many entities all defined by the presence of benign tumours arising from uterine smooth muscle cells. The authors aim through this case report to address the underdiagnosis of IVL in pre-menopausal women and the potential confusion with other cardiac tumours.

CASE PRESENTATION

A 48-year-old woman initially treated for a suspected intracardiac myxoma underwent two surgeries. Seeking gynaecological care for menometrorrhagia related to a polymyomatous uterus, she was scheduled for radical surgery. Unexpectedly, extended explorations during a hysterectomy revealed a tumour originating from the pelvis, extending to the right atrium and inferior vena cava, indicative of intravascular leiomyomatosis.

CLINICAL DISCUSSION

IVL's diagnosis is often incidental during hysterectomy analysis, with symptoms mimicking uterine fibroids. Treatment involves radical surgery, emphasizing the importance of complete resection to reduce the significant risk of recurrence.

CONCLUSION

Pre-menopausal women with a history of hysterectomy or myomectomy and a detected right chamber mass should be screened for intravascular leiomyomatosis. Diagnosis relies on histological examination, guiding tailored treatment choices such as surgical resection with a focus on bilateral adnexectomy for optimal outcomes.

摘要

引言与重要性

静脉内平滑肌瘤病(IVL)是一组已知的异位平滑肌瘤病中的一种病理类型,该组疾病包含许多实体,均由源自子宫平滑肌细胞的良性肿瘤的存在所定义。作者旨在通过本病例报告探讨绝经前女性中IVL的诊断不足以及与其他心脏肿瘤的潜在混淆。

病例介绍

一名48岁女性最初因疑似心脏黏液瘤接受了两次手术。因多发性子宫肌瘤导致月经过多而寻求妇科护理,她被安排进行根治性手术。意外的是,子宫切除术中的广泛探查发现了一个起源于盆腔、延伸至右心房和下腔静脉的肿瘤,提示血管内平滑肌瘤病。

临床讨论

IVL的诊断通常在子宫切除术分析过程中偶然发现,症状类似子宫肌瘤。治疗包括根治性手术,强调完全切除以降低复发的重大风险的重要性。

结论

有子宫切除术或肌瘤切除术史且检测到右心房肿块的绝经前女性应筛查血管内平滑肌瘤病。诊断依赖于组织学检查,指导如手术切除等量身定制的治疗选择,重点是双侧附件切除术以获得最佳结果。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/05e7/10923348/5c27e65a3681/ms9-86-1766-g001.jpg

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