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)
References
1. Kurman R.J., Hedrick E.L., Ronnett B.M. (eds). Blaustein’s Pathology of the Female Genital Tract. 7th ed. Springer, 2019. 1508 p.
2. Mandelbaum I., Pauletto F.J., Nasser W.K. Resection of a leiomyoma of the inferior vena cava that produced tricuspid valvular obstruction. J Thorac Cardiovasc Surg. 1974; 67 (4): 561-7.
3. Iverson L.I., Lee J., Drew D., et al. Intravenous leiomyomatosis with cardiac extension. Tex Heart Inst J. 1983; 10 (3): 275-8.
4. Stilidi I.S., Payanidi Yu.G., Bokhyan V.Yu., Zakharova T.I., Zhordania K.I. Intravenous leiomyomatosis (description of a case). Onkoginekologiya [Oncogynecology]. 2012; (1): 47-50. (in Russian)
5. Li B., Chen X., Chu Y.D., et al. Intracardiac leiomyomatosis: a comprehensive analysis of 194 cases. Interact Cardiovasc Thorac Surg. 2013; 17 (1): 132-8. DOI: https://doi.org/10.1093/icvts/ivt117
6. Clay T.D., Dimitriou J., Mcnally O.M., et al. Intravenous leiomyomatosis with intracardiac extension - a review of diagnosis and management with an illustrative case. Surg Oncol. 2013; 22 (3): e44-52. DOI: https://doi.org/10.1016/j.suronc.2013.03.004
7. Ma G., Miao Q., Liu X., et al. Different surgical strategies of patients with intravenous leiomyomatosis. Medicine (Baltimore). 2016; 95 (37): e4902. DOI: https://doi.org/10.1097/MD.0000000000004902