J Huazhong Univ Sci Technol［Med Sci］ 31(4):2011

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Intravenous Leiomyomatosis with Right Heart Involvement— A Report of 4 Cases and Literature Review

Yiqing LI (李毅清), Fei MEI (梅 菲), Chao YANG (杨 超)#, Ping LV (吕 平), Chenxi OUYANG (欧阳晨曦), Bi JIN (金 毕) Department of Vascular Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan

430022, China © Huazhong University of Science and Technology and Springer-Verlag Berlin Heidelberg 2011

Summary: Intravenous leiomyomatosis (IVL) is a rare benign neoplasm which originates from the smooth muscle cells and is usually confined to the pelvic venous system. Rarely, intracaval and in-tracardiac extension has been described. Death can occur as a result of intracardiac involvement. We reported 4 cases of IVL with right heart involvement (intracardiac leiomyomatosis, ICL). Three of them suffered recurrent sudden syncope, and the other one was totally asymptomatic. All of them were suc-cessfully treated through one-stage operation under extracorporeal circulation. Key words: intravenous leiomyomatosis; intracardiac leiomyomatosis; cardiac tumor; surgical man-agement

Intravenous leiomyomatosis (IVL) is a rare benign neoplasm, which usually originates from the smooth muscle cells of uterus and grows within pelvic venous system and inferior vena cava, even involving the heart. When growing into the heart the IVL is called intracar-diac leiomyomatosis (ICL)[1]. Although histological be-nign, IVL might behave clinically in a “malignant” way. Sudden death can occur as a result of ICL with subse-quent obstruction of the venous return to the heart. There is only one leiomyoma body growing within the vein in most of the cases reported by the literature so far. How-ever, one of the four cases was found to have two leio-myoma bodies: one extended from left internal iliac vein into the heart; the other was much smaller, which grew in a branch of inferior vena cava between the two internal iliac veins.

1 CASE REPORT

Case 1: A 36-year-old woman, who was admitted because of sudden and transient syncope for one time. She had no special past history. Physical examination revealed a heart rate of 80 beats/min in regular sinus rhythm and a grade 4/6 systolic murmur was present at left sternal border. The CT scan confirmed the presence of thrombotic material in the right chambers of the heart and the inferior vena cava, with extensive thrombosis extending until the confluence of the common iliac veins and further thrombotic material inside the right internal iliac vein. Moreover, fibromatosis of the uterus and mul-tiple cystic lesions in the right periovarian region were also found.

Case 2: A 48-year-old woman, who was admitted because of abdominal mass for 2 months. She also suf-

Yiqing LI, E-mail: qzg599@126.com #Corresponding author, E-mail: chaoyang83@163.com

fered sudden syncope for several times but without any special past history. Physical examination revealed a heart rate of 86 beats/min in regular sinus rhythm and a grade 2-3/6 systolic murmur was present at left sternal border. A moderately hard and fixed mass could be palpated in lower abdomen. Type B ultrasound exam performed at our hospital revealed a large mass in pelvic cavity with closed relationship to left lateral wall. Ultrasonic cardio-gram revealed a mobile mass extending from the inferior vena cava into the right atrium and ventricle, without evidence of attachment to the right atrial or ventricular walls. The MRI was also taken with the result of a con-tinuous mass extending from left internal iliac vein to right atrium.

Case 3: A 51-year-old woman, who was admitted because of recurrent sudden syncope for 5 months and worsens for one week. During the past 5 months, she suffered from sudden unconsciousness several times when walking or changing position suddenly, after a rest, she could recover by herself. She had a history of hys-terectomy because of hysteromyoma. Physical examina-tion revealed a heart rate of 66 beats/min in regular sinus rhythm and a grade 2/6 systolic murmur was present at left 2-4 intercostal space of left sternal border. The MRI confirmed abnormal signal in right iliac veins, inferior vena cava and right atrium, considered as tumors. Dy-namic MRI showed that the mass of abnormal signal in the atrium moved through the outflow tract of right atrium into right ventricle during the ventricular diastolic phase, and back during systolic phase.

Case 4: A 43-year-old woman, who was admitted with incidental evidence of a pelvic intracaval mass ex-tending to the right atrium, and was totally asymptomatic. She had a history of leiomyoma of the uterus. Physical examination revealed a heart rate of 104 beats/min in regular sinus rhythm without any murmur. The MRI con-firmed abnormal signal in right iliac veins, inferior vena cava and right atrium, considered as tumors, and there

31(4):586-588,2011 J Huazhong Univ Sci Technol［Med Sci］ DOI 10.1007/s11596-011-0494-0

J Huazhong Univ Sci Technol［Med Sci］ 31(4):2011

587

was a large mass in pelvic cavity. The surgical procedures were all performed by De-

partment of Vascular Surgery together with Department of Cardiac Surgery. At surgery, extracorporeal circula-tion was established after the median sternotomy, at the same time, the inferior vena cava was exposed and iso-lated by laparotomy. After opening the inferior vena cava and transsection, the masses were pulled out completely

by steady force from right artrium. Then we explored the attached branch of inferior vena cava, found the attach-ment point of the masses and resected them. For the cases 1, 2 and 4, the masses in pelvic were resected by one-stage-surgery. Operations were successful and there were no complications. IVL was confirmed pathologi-cally (fig. 1).

Fig. 1 A: The ophicephalous-form tumor in right atrium, right ventricle and inferior vena cava; B: A mobile mass extending from the

inferior vena cava into the right atrium and ventricle; C: Surgical resection specimen: Snake-like IVL body; D: Pathologic find-ing of IVL (HE staining ×200)

In the cases 1, 2 and 4, only one mass was detected.

In the case 3, two masses were found: the larger one, a length of 35 cm, light pink color, integrated amicula and moderate hardness; the smaller one in a branch of IVC, between the two internal iliac veins. In order to prevent recurrence the left internal iliac vein and another brunch of IVC were ligated, in which the smaller mass was found. 2 DISCUSSION

IVL is a rare benign tumor, which was first reported

by Birch-Hirschfeld in 1896 and since then many reports have dealt with IVL. For recent 10 years, the literature contains more reports of IVL, fewer than 100 cases with cardiac involvement[2]. Harris reported that 64% of pa-tients with IVL had a history of hysterectomy, diagnosed after 4 years[3]. The tumor is characterized by the growth of smooth muscle tumor cells into venous channels, which penetrates to the inferior vena cava and reaches the right atrium. Two mechanisms have been provided: It’s due to the myomas of the uterus which attack the vessels extensively; Besides, some tumors can originate from the smooth muscle of the veins[4, 5]. 2.1 Diagnosis

IVL has been rarely described in surgical, gyneco-logical, and radiological reports; but potential extension into the right-sided cardiac cavities could bring on sud-den death. With the unspecific symptoms, complex clinical manifestations and slow growing speed, IVL is not easy to be detected, and it’s difficult to get the correct diagnosis before the operation[6]. Correct diagnosis de-pends on the sufficient recognition. IVL should be con-sidered in young women with tumors in uterus or peri-uterus accompanied by clues of deep phlebothrombosis or tumors in the right atrium and vena cava[7]. The clini-cal manifestations may not appear until the right heart is involved in most cases, such as cardiogenic syncope, panicky, edema of lower extremity, hepatomegalia, as-cites and cardiac murmur, which is easy to be considered as cardiac primartumor[3, 7]. Just as the cases 1 and 3, the first symptom is only repeated syncope, so it is always

considered as disease of nervous system. With regard to this, the echocardiography has important screening value. We see echogenic mass of low-level echo, the attachment point of the mass always on the wall of inferior vena cava not on the wall of atrium, and the inferior vena cava is widen with obturator in the cave; However, myxoma of the right atrium is seen as echogenic mass of high-level echo, the range of motion is wider and the root of mass is found on the interauricular septum. Besides, the CT scan can detect the enlargement of the inferior vena cava and the soft tissue density mass in the cavity, and the mass in the uterus and pelvic cavity is linked to the one in the iliac vein. All these findings will be helpful to diagnose IVL[7, 8]. MRI test clearly display the worm-like signal of the tumor in the inferior vena cava, and the ophicephalous-form tumor in the right atrium when it is involved, superior margin dissociated and in-ferior margin stretched to the focus in inferior vena cava. MRI test can not only identify the range of disease and the character of the tumor, but also can show us whether there is adherence and where the adherence is, which are significantly important for the surgery.

Diagnosis relies on the postoperative pathological test of the tumor. Under the gross examination, generally the snake-like tumor in inferior vena cava does not ad-here to the vessel wall. Under the microscopy, the benign smooth muscle bundles encroach into the venous cavity and form a large lobulated embolus occupying the ve-nous cavity. There are muscular vessels with thrombus and red cells in them, proving the one invaded by the tumor is not lymphatic duct. The tumor in the big vessel is covered by blood vessel endothelium and endomem-brane, which suggests that the tumor intrude through the vessel wall, covered by the endothelium, growing to-gether with the endothelium along the blood flow[4, 5]. 2.2 Treatment

Surgery is the treatment of choice, and the complete removal of the tumor and of the thrombus is mandatory because recurrence is frequent despite the benign nature of the neoplasm. Different surgical techniques have been utilized for this purpose: removal of the intracardiac mass through the iliac vein or through the right atrium,

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performed with a one- or double-stage procedure, with extracorporeal circulation or during a period of circula-tory arrest, has been proposed[1, 9]. In our cases, we chose a one-stage strategy. For double-stage procedure, the first stage is thoracotomy with extracorporeal circulation, low perfusion with hypothermia, even ceasing circulation with profound hypothermia, pulling the tumor out from the incision of the right atrium. The second stage is ab-dominal surgery. Because this disease is usually origi-nated from uteral muscular veins, the hysterectomy should be given as soon as possible. Preoperative imag-ing of vessels is necessary to detect the tumor in uterus or the bilateral appendages, also the pathway of the tu-mor in veins[1, 10, 11]. 2.3 Prevention of Recurrence

Case 3 indicates the existence of double-tumor form, therefore, during the surgery, the attachments should be found as far as possible, and resected together with the endomembrane to prevent recurrence, if necessary, all the branches of inferior vena cava should be explored. As reported, the tumor can progress along the iliac vein or vena ovarica into the inferior vena cava in 79.4% of the cases, and most of these cases have one-side growth, seldom both. Of which, 47.1% grow along the iliac vein, 25% along the vena ovarica into the renal vein and 5% along both[11]. When the attachment point is lower than the iliac vein, the ligation of iliac vein would be consid-ered, which has been performed in our cases and recur-rence hasn’t been detected by type-B ultrasonic examina-tion till now.

Some researchers suggest that this disease is due to estrogen, since estrogenic receptors are found in the tu-mors which invade the vena cava and right atrium. Therefore, they consider antioestrogens as adjunctive therapy, which will be effective to prevent progress and recurrence[12]. 2.4 Conclusion

In a word, the treatment key point of IVL is early discovery and prevention of recurrence. It requests sur-geons to recognize this disease sufficiently to get correct diagnosis. IVL should be considered in young women with symptoms of cardiac inadequacy and a right-sided intracardiac mass, particularly when hysterectomy for leiomyoma was previously performed. Evidence can be detected by type-B ultrasonic examination, CT or MRI, and the tumor can be resected completely.

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(Received June 30, 2011)