Intravenous Leiomyomatosis

Published on 10/03/2015 by admin

Filed under Obstetrics & Gynecology

Last modified 22/04/2025

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Intravenous Leiomyomatosis

Synonyms/Description

Benign metastasizing leiomyoma

Etiology

This is caused by intravascular proliferation of a smooth muscle, leiomyoma-like tumor, which is noninvading locally, but grows within venous channels of the uterus and pelvis as serpiginous tubular masses. This growth can be extensive and occasionally even reach the inferior vena cava and the right atrium, causing cardiac symptoms. In one study, 56% of patients with this condition had previously had a hysterectomy for uterine fibroids.
There are two possible etiologies for this rare tumor: (1) The tumor may originate from smooth muscle in the vessel wall itself. (2) The tumor originates from a uterine leiomyoma, subsequently invading adjacent venous channels.

Ultrasound Findings

These leiomyoma-like lesions are nodular, tubular, and serpiginous solid masses in the pelvis. The intravascular location of these masses is not well seen sonographically. Color flow is typically visible within these masses. The characteristic features are tubular solid masses, often bilateral, following the course of pelvic veins.

Differential Diagnosis

Pelvic malignancies separate from the uterus and ovaries, such as sarcoma or lymphoma.

Clinical Aspects and Recommendations

This is an extremely rare condition. The disease is usually asymptomatic and will often be discovered incidentally on a chest x-ray. However, some patients will present with a cough, shortness of breath, or even chest pain. The ultimate diagnosis is based upon histologic findings of the extrauterine leiomyomas on bronchoscopic biopsy.
When the disease is asymptomatic, expectant management may be an option, although the treatment of choice for symptomatic patients is removal of the intravascular tumors as well as hysterectomy/bilateral salpingo-oophorectomy. The tumor typically contains estrogen and progesterone receptors. Thus treatment with oophorectomy and medications that induce medical menopause, such as GnRH agonists, aromatase inhibitors, or even progestins, has been shown to result in tumor regression. Usually the lesions will shrink or disappear after castration. Thus, hormone replacement therapy is usually contraindicated.

Figure

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Figure I2-1 Four different views of the serpiginous and tubular solid mass in the right side of the pelvis, which was subsequently proven to be intravenous leiomyomatosis. There was abundant Doppler color flow in the mass.

Suggested Reading

Andrade L.A., Torresan R.Z., Sales Jr. J.F., Vicentini R., De Souza G.A. Intravenous leiomyomatosis of the uterus. A report of three cases. Pathol Oncol Res.. 1998;4:44–47.

Diakomanolis E., Elsheikh A., Sotiropoulou M., Voulgaris Z., Vlachos G., Loutradis D., Michalas S. Intravenous leiomyomatosis. Arch Gynecol Obstet. 2003;267:256–257.

Kokawa K., Yamoto M., Yata C., Mabuchi Y., Umesaki N. Postmenopausal intravenous leiomyomatosis with high levels of estradiol and estrogen receptor. Obstet Gynecol. 2002;100:1124–1126.

Lou Y.F., Shi X.P., Song Z.Z. Intravenous leiomyomatosis of the uterus with extension to the right heart. Cardiovasc Ultrasound. 2011;24(9):25.

Low G., Rouget A.C., Crawley C. Intravenous leiomyomatosis with intracaval and intracardiac involvement. AJR. 2012;265:971–974.

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