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

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.