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表现为右心室肿块的良性转移性平滑肌瘤

Benign Metastasizing Leiomyoma Presenting as a Right Ventricular Mass.

作者信息

Romano Edoardo, Schütze Jonathan, Schönhoff Florian, Siegenthaler Franziska, Banz Yara, Gräni Christoph

机构信息

Department of Cardiology, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland.

Department of Cardiac Surgery, Inselspital Bern, University Hospital Bern, Bern, Switzerland.

出版信息

JACC Case Rep. 2025 Sep 3;30(26):104914. doi: 10.1016/j.jaccas.2025.104914.

Abstract

BACKGROUND

Masses in the right ventricle are uncommon, but if present, they are most often attributed to either primary cardiac tumors or metastatic disease.

CASE SUMMARY

A 50-year-old woman presented with progressive lower extremity edema and was diagnosed with a right ventricular mass causing severe tricuspid insufficiency and near-total obstruction of the pulmonary artery. She had a history of hysterectomy for uterine leiomyomatosis. Based on imaging and histological findings, a diagnosis of benign metastasizing leiomyoma (BML) was made. The patient underwent successful resection of the mass, tricuspid valve replacement, and implantation of a right ventricle-pulmonary artery conduit.

DISCUSSION

Cardiac metastasis from BML is extremely rare, particularly in the right heart. Although histologically benign, BML can lead to life-threatening cardiac complications. This case emphasizes the importance of thorough cardiac assessment in patients with a history of leiomyoma and unexplained symptoms. Early diagnosis and surgical management are essential to prevent serious outcomes.

TAKE-HOME MESSAGES: BML, though rare, can cause life-threatening cardiac involvement. Clinicians should consider a comprehensive cardiac evaluation for patients with unexplained cardiac symptoms and a history of leiomyoma.

摘要

背景

右心室肿块并不常见,但如果存在,最常见的原因是原发性心脏肿瘤或转移性疾病。

病例摘要

一名50岁女性因进行性下肢水肿就诊,被诊断为右心室肿块,导致严重三尖瓣关闭不全和肺动脉几乎完全阻塞。她有因子宫平滑肌瘤病行子宫切除术的病史。根据影像学和组织学检查结果,诊断为良性转移性平滑肌瘤(BML)。患者成功切除肿块,置换三尖瓣,并植入右心室-肺动脉导管。

讨论

BML的心脏转移极为罕见,尤其是在右心。尽管组织学上为良性,但BML可导致危及生命的心脏并发症。该病例强调了对有平滑肌瘤病史且有不明原因症状的患者进行全面心脏评估的重要性。早期诊断和手术治疗对于预防严重后果至关重要。

要点

BML虽然罕见,但可导致危及生命的心脏受累。临床医生应对有不明原因心脏症状且有平滑肌瘤病史的患者进行全面的心脏评估。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/db1e/12426687/c1f2d4a83cd6/ga1.jpg

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