Uterine intravenous leiomyomatosis with an isolated large metastasis to the right atrium: a case report
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An intravenous leiomyomatosis is a special type of uterine leiomyoma characterized by the formation of benign leiomyomatous tissue within the vascular wall. Although histologically benign, intracardiac metastasis can lead to circulatory failure, and death, if untreated. Herein, we report on a case of a uterine intravenous leiomyomatosis with an isolated large adherent metastasis in the right atrium of the heart.
A 52-year-old Japanese woman sought medical attention at our hospital for lower abdominal pain. A 27-cm uterine mass was detected on clinical imaging, with a 78 × 47-mm mass in the right atrium detected on preoperative echocardiography. Intracardiac mass resection and tricuspid annuloplasty were performed as the first-stage surgery. The pedicle of the tumor was adherent to the wall of the atrium. On histological examination, the tumor was found to consist of spindle-shaped cells with eosinophilic cytoplasm, without atypia, but with a myxoid change, and rich microvascularization of the pedicle. Total abdominal hysterectomy was performed as the second-stage surgery, with confirmation of the diagnosis as uterine intravenous leiomyomatosis with an isolated metastasic lesion to the right atrium. There has been no evidence of tumor recurrence in the 15 months since surgery.
We report a unique case in which a large right atrial leiomyoma was identified following a uterine intravenous leiomyomatosis. Our case exemplifies that intravenous leiomyomatosis metastatic tumors have the potential to grow via their vascularization.
KeywordsUterus Intravenous leiomyomatosis Cardiac metastasis Right atrium Case report
Magnetic resonance imaging
α-smooth muscle actin
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 . IVL with intracardiac involvement was first reported in 1907, with fewer than 300 cases presently documented in the literature, since then . Although histologically benign, intracardiac IVL extension can lead to circulatory failure or death if left untreated . 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 . Some studies have reported on isolated intracardiac tumor from uterine IVL that are adherent to the cardiovascular wall [5, 6, 7, 8]. Herein, we report such a case of uterine IVL that clinicopathologically preceded IVL metastasis into the right atrium.
The patient provided consent for publication of this case report.
Gross and microscopic findings
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 .
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.  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.
Cardiac metastasis of intravenous leiomyomatosis
Present case (2018)
Anterior wall of RA
TR + TH + TVP
Thukkani et al., (2005) 
TH + USO for IVL
TR + USO + TVP
(at 26 years)
Baboci et al., (2014) 
TH for IVL
Shortness of breath
Anterior wall of RA,
TR + lung
(at 47 years)
TV, chordae tendineae
Lin and Liu, (2014) 
TH + BSO for IVL
(at 42 years)
Zhang and Lang, (2016) 
TR + TH + BSO
+TVP + GnRHa
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.
We would like to thank Editage (www.editage.jp) for English language editing.
MiY: conceptualization, pathologic diagnosis, immunohistochemical analysis, and writing of manuscript. YN, SI: collection of clinical data. TK, RK: pathologic diagnosis and immunohistochemical analysis. EK: radiologic analysis. MaY (the last author): pathologic diagnosis and revision of manuscript. All authors read and approved the final manuscript prior to submission.
This work was supported by Sekiguchi Memorial Award (grant no. 18-C-1-02) at Saitama Medical University and Grants-in-Aid from the Ministry of Education, Science, Sports and Culture of Japan (Research Project Numbers: 18 K06997).
Ethics approval and consent to participate
The patient provided consent for publication of the case report.
Consent for publication
Written informed consent was obtained from the patient for the publication of this case report.
The authors declare that they have no competing interests.
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