Background
Intravenous leiomyomatosis (IVL) is a special type of uterine leiomyoma, characterized by the formation of benign leiomyomatous tissue within the vascular vessels of the uterus. The tumor typically grows along vascular vessels and, thus, can extend to the iliac vein, inferior vena cava and, even to the heart. IVL develops in only 0.1% of women with uterine leiomyomas, with intracardiac involvement identified in 10–40% of IVL cases [
1]. IVL with intracardiac involvement was first reported in 1907, with fewer than 300 cases presently documented in the literature, since then [
2]. Although histologically benign, intracardiac IVL extension can lead to circulatory failure or death if left untreated [
3]. The typical features of IVL include contiguous pelvic and intravenous masses, with sausage-shaped lesions in the inferior vena cava, whereas intracardiac tumors arising from IVL typically show a worm-like appearance [
4]. Some studies have reported on isolated intracardiac tumor from uterine IVL that are adherent to the cardiovascular wall [
5‐
8]. Herein, we report such a case of uterine IVL that clinicopathologically preceded IVL metastasis into the right atrium.
Discussion and conclusions
IVL has the potential to grow along blood vessels, extending to the iliac vein, inferior vena cava, and even to the heart. Typically with IVL, the pelvic and intravenous masses are continuous and the intravenous lesions do not invade or adhere to the vessel wall [
4].
In the present case, however, the intracardiac metastasis arising from the uterine IVL was isolated and was adherent to the endocardium of the atrium, with no evidence of tumor occurrence in the inferior vena cava, internal iliac vein or ovarian vein. The tumor of the right atrium was an independent metastasis of the uterine IVL, mimicking a primary cardiac myxoma. In the presence of uterine IVL, it is necessary to distinguish the nature of the intracardiac mass, regardless of the absence of its continuity with the uterus. Of note, the intracardiac tumor in the present case was larger than the diameter of the inferior vena cava and, thus, was considered to have developed within the heart. This differs from the report by Maneyama et al. [
9] in which a spontaneous migration of a residual IVL to the heart, via the blood stream after hysterectomy, was described. The intracardiac tumor of the present case was adherent to the wall of the right atrium wall via a richly vascularized pedicle. This microvasculature is what likely allowed the tumor to grow.
A summary of previous reports [
5‐
8] on the occurrence of an isolated cardiac metastasis from uterine IVL is provided in Table
1. The tumor diameter of the present case was more than twice that of previously reported cases. The intracardiac mass was identified after hysterectomy for IVL in 3 of 5 cases, with uterine fibroids identified in the other 2 cases. Of note, in all previously reported cases, there was concurrent evidence of lung metastases, which was not the case for our patient. Tumor progression after surgery was not identified in any of these previous cases. Ordulu et al. [
10] reported the expression of HMGA2 protein, a driver for tumor metastasis [
11], in 58% of IVL cases, which is higher than the 32% in cases with typical uterine leiomyoma. Regional chromosomal alterations of variable frequencies were also observed in IVL, showing overlaps with uterine leiomyosarcoma [
12]. Thus, IVL has an intermediate biological propensity between a benign and malignant status. Recurrence of IVL tends to occur in younger patients. Du et al. [
1] have suggested that young patients should be treated using hysterectomy and salpingo-oophorectomy if the patient does not wish to maintain fertility. Mizoguchi et al. [
13] pointed out that anti-estrogen therapy may be an effective treatment if the patient has not yet entered menopause. These results suggest that IVL treatment requires tumor reduction and blockage of blood flow and estrogen to limit continued growth of the tumor and possible metastasis.
Table 1Cardiac metastasis of intravenous leiomyomatosis
Present case (2018) | 52 | Uterine leiomyoma | Abdominal pain | 78 | Anterior wall of RA | Heart | TR + TH + TVP | PFS |
| | | | | | +GnRHa | (14 months) |
Thukkani et al., (2005) [ 5] | 36 | TH + USO for IVL | Abdominal swelling | 15 | TV | Heart, lung | TR + USO + TVP | PFS |
| (at 26 years) | | | | | +GnRHa | (12 months) |
Baboci et al., (2014) [ 7] | 51 | TH for IVL | Shortness of breath | NA | Anterior wall of RA, | Heart, lung | TR + lung | PFS |
| (at 47 years) | | | TV, chordae tendineae | | segmentectomy | (2 years) |
| 43 | TH + BSO for IVL | Palpitation, | 15 | TV | Heart, lung | None | PFS |
| (at 42 years) | chest distress | | | | | (7 years) |
Zhang and Lang, (2016) [ 6] | 40 | Uterine mass | None | 30 | Chordae tendineae, | Heart, lung | TR + TH + BSO | PFS |
| | | | papillary muscles | | +TVP + GnRHa | (2 months) |
In conclusion, we report a unique case in which a right atrial leiomyoma was identified following a uterine leiomyoma. The uterine IVL had an isolated large metastasis to the right atrium that was adherent to the endocardium via a richly vascularized pedicle. Our case exemplifies that IVL metastatic tumors have the potential to grow via their vascularization.
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