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Published on: February 14, 2017
Intracardiac leiomyomatosis: echocardiographic features
I J Kullo1, J K Oh, G L Keeney
1Division of Cardiovascular Diseases and Internal Medicine, Mayo Clinic and Mayo Foundation, Rochester, Minnesota 55905, USA.
Insights
Intravenous leiomyomatosis, a benign smooth-muscle tumor, can extensively spread to the heart. Echocardiography reveals characteristic masses in the cardiac chambers and pulmonary arteries.
Area of Science:
- Cardiology
- Oncology
- Pathology
Background:
- Intravenous leiomyomatosis (IVL) is a rare condition characterized by benign smooth-muscle tumors extending through vascular channels.
- It typically arises from uterine fibroids or uterine vessels, often presenting years after hysterectomy.
Observation:
- This study describes two middle-aged women with a history of hysterectomy who presented with cardiac symptoms.
- Echocardiography revealed extensive IVL involving the right-sided cardiac chambers and pulmonary arteries, originating from the inferior vena cava and azygos vein.
Findings:
- Distinctive echocardiographic findings included elongated, mobile masses within the venous system and heart.
- Multiple venous attachments and filling of venous vessels and right-heart chambers were observed, indicating tumor spread.
Implications:
- Intracardiac leiomyomatosis should be considered in the differential diagnosis for women presenting with cardiac masses.
- Echocardiography is crucial for identifying the extent of IVL within the cardiovascular system and guiding management.
Abstract:
Intravenous leiomyomatosis is a histologically benign smooth-muscle tumor arising from either a uterine myoma or the walls of a uterine vessel with extension into veins. Echocardiographic features of two cases of intravenous leiomyomatosis with extensive spread into the right-sided cardiac chambers and pulmonary arteries are described. Both patients were middle-aged women, with prior history of hysterectomy 12 and 10 years earlier who presented with cardiac symptoms and signs. Distinctive echocardiographic features include 1) elongated mobile masses extending from the veins of the lower body, including inferior vena cava and azygos vein; 2) multiple venous attachments or metastases; and 3) filling of venous vessels and right-heart chambers. Intracardiac leiomyomatosis should be considered in a female patient presenting with an extensive mass in the right-sided cardiac chambers.
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