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1 . 2021

Intracardiac intravenous leiomyomatosis: description of a clinical case

Abstract

Intravenous leiomyomatosis with intracardiac extension is a rare entity characterized by growth of a uterine neoplasm that extends through the uterine vascular channels through the venous system and ultimately into the cardiac chambers. The tumors may lead to significant physiologic disturbances and heart failure. In case when tumor thrombus reaches pulmonary arteries severe pulmonary embolism can occur. In some cases, cardiac arrest may occur, particularly when the tumor involves the cardiac chambers. Histogenesis of intravenous leiomyomatosis has not been sufficiently explored. Currently there are two major theories of tumor histogenesis. According to one of them, leiomyoma of the uterus sprouts a wall of small venules that contributes to further intravascular growth. According to the other one, intravenous leiomyomatosis originates from venous wall muscle cells. Yet the majority of authors support the theory stating that intravenous leiomyomatosis arises from uterine myometrium for the following reasons. First, in most cases patients have a medical history of leiomyoma of the uterus. Second, the tumor is often directly associated with pelvic tumors, and herewith, it has no obvious adhesion with the venous wall and is free-floating in the lumen of the veins. Third, intravenous leiomyomatosis has estrogen and progesterone receptors which is uncommon for myocytes of venous wall.

Intravenous leiomyomatosis commonly occurs in premenopausal women and approximately 83% have undergone a previous hysterectomy or myomectomy. Clinical picture of intravenous leiomyomatosis is non-specific and diverse. Mostly clinical manifestations occur when a tumor extends into cardiac cavities. Some patients with intracardiac intravenous leiomyomatosis didn't have any symptoms of the disease due to normal venous return. In other patients with cardiac lesions cardiac symptoms prevailed. In case when tumor thrombus reaches pulmonary arteries severe pulmonary embolism can occur. Literature described the cases of cardiac arrest in patients with intracardiac intravenous leiomyomatosis. Radical surgery is currently the standard treatment. This patient with intravenous leiomyomatosis which spread into the right atrium and right ventricle.

Conclusion. Intravenous leiomyomatosis with intracardiac spread is a rare disease of a challenging localization, that require performing of complicated surgical interventions, excellent technical equipment and technology infrastructure of the operating room and multidisciplinary cooperation, that is possible to provide only in highly specialized (tertiary) multipurpose medical institutions.

Keywords:intracardiac intravenous leiomyomatosis, uterine leiomyoma, smooth muscle tumor of uncertain malignant potential

Funding. The study had no sponsor support.
Conflict of interests. The authors declare no conflict of interests.
For citation: Stilidi I.S., Charchyan E.R., Zhordania K.I., Bokhian V.J., Kozlov N.A., Pajanidi J.G. Intracardiac intravenous leiomyomatosis: description of a clinical case. Clinical and Experimental Surgery. Petrovsky Journal. 2021; 9 (1): 70-6. DOI: https://doi.org/10.33029/2308-1198-2021-9-1-70-76 (in Russian)

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CHIEF EDITOR
CHIEF EDITOR
Sergey L. Dzemeshkevich
MD, Professor (Moscow, Russia)

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