Article Type : Case Report
Authors : Alizadeasl A, Hoseini S, Bahramnejad A, Dousti A, Roudini K, Mohseni M, Baghai-wadji M, Tahooni A, and Afsari Y
Keywords : Intravascular leiomyomatosis; Intracardiac leiomyomatosis
Intra vascular spread of leiomyoma from
uterus to the heart is a rare condition in which proliferation and growth of
smooth muscle cells from uterus extends via vascular root and the main
treatment is surgery. Here we present a case of Intra-vascular leiomyomatosis
who presented with cardiac mass.
Leiomyoma is a benign tumor of uterus which is made of
smooth muscle cells proliferation [1]. This tumour is usually localized in the
uterus but as a rare condition it could proliferate and extend via intravenous
root and invade the heart via inferior Vena Cava (IVC) [2]. Here we want to
present a case of Intravenous leiomyomatosis who presented to our hospital with
dyspnea and leg edema.
A 50-year-old lady without any remarkable past medical
history presented to our hospital with complaint of shortness of breath and
intermittent lower limb edema in the past 2 months ago, she had familial
history of metastatic peritoneal cancer with unknown origin in her mother, at
presentation her vital signs and hemodynamics were stable. As primary
evaluation chest X-ray and trans-thoracic echocardiography (Figure 1) was done
which showed normal Left Ventricle (LV) size and systolic function(LVEF:55%), mild
Right Ventricle (RV) enlargement and dysfunction, normal Left Atrium (LA) size,
severe Right Atrium (RA) enlargement, mild Mitral Regurgitation (MR), mild
Pulmonary Insufficiency (PI), Normal Tri-cuspid Valve, with at least moderate
Tricuspid Regurgitation (TR) (TR Gradient: 20 mmHg), SPAP: 35 mmHg, Increased
Inferior Vena Cava (IVC) size with No collapse, turbulent flow was seen in
ostium of IVC with no significant gradient. So the patient underwent
Trans-Esophageal Echocardiography (TEE) (Figure 2). Which showed large mobile
and well differentiated RA mass (7.8cm x 3.5cm) originated from IVC with
multiple mobile particles on it, that some of them were calcified, and another
mass was seen on anterior leaflet of Tri-Cuspid Valve (1 x 0.8 cm) with no significant
Tri-Cuspid Stenosis (TS). In this stage two differential diagnosis was made: a
metastasis to the heart or thrombosis; so Intra-Venous (IV) heparin infusion
started and so search for origin of possible metastasis. In abdomino-pelvic
sonography, a heterogenous isoechoic mass measured 16 x 10 mm of uterine origin
with right adnexa involvement is involved suggestive for sarcoma, accompanied
tumour-thrombosis extending from right ovarian vein to IVC and eventually to
the right atrium was reported. Therefore, uterine mass biopsy performed which
pathology reported submucosal leiomyoma; and also, Cardiac Magnetic Resonance
(CMR) (Figure 3).
Figure 1: Trans-thoracic echocardiography; the right image shows the mass in right atrium of the heart and the left image shows the mass in inferior Vena cava.
Figure 2: Trans-esophageal echocardiography of the mass in the heart.
Figure 3: Cardiac MRI of the mass in the heart.
Figure 4: MRI of the mass in the inferior Vena cava.
Imaging performed which showed RA enlargement, Severe
TR (Regurgitation Fraction > 30%), no myocardial edema or scar, bulky large
mass in IVC (Figure 4). originated from right gonadal vein with extension to RA
(bulk of tumour 79 x 38 mm) with partially perfusion, all suggestive for tumour
or thrombosis originated from pelvic, other possibility as intra-vascular
extension of uterine leiomyoma could be considered as differential diagnosis.
The patient underwent simultaneous laparotomy and thoracotomy for total
hysterectomy and bilateral salpingo-oophorectomy and cardiac mass resection and
the mass sent for pathology which reported leiomyomatosis; after 6 months
another CMR performed which showed no residual mass and the patient is under
follow-up.
Leiomyoma is a primary tumour of uterus, and is
consistent of smooth muscle cells proliferation which rarely could extent via
uterine or adnexal veins to IVC and make intra-vascular leiomyomatosis (IVL)
and intra-cardiac leiomyomatosis (ICL) [3]. The main treatment is radical
excision and follow up in 6 months and then 2-5 years later is strongly
recommended [4]. There is multiple imaging modality to diagnose and follow up
ICL like echocardiography, enhanced CT and MRI which can demonstrate anatomy
and physiology of the heart and so the mass [5].
The authors have nothing to disclose.
There was no funding.