Ann Thorac Surg 2004;78:330-332
© 2004 The Society of Thoracic Surgeons
Case report
Intravenous leiomyomatosis extending into the right ventricle after subtotal hysterectomy
Mehmet Sah Topcuoglu, MDa*,
Hafize Yaliniz, MDa,
Hakan Poyrazoglu, MDa,
Acar Tokcan, MDa,
Süleyman Cansun Demir, MDb,
Abdi Bozkurt, MDc,
Handan Zeren, MDd
a Cardiovascular Surgery, Medical School, Çukurova University, Adana, Turkey
b Obstetrics and Gynecology, Medical School, Çukurova University, Adana, Turkey
c Cardiology, Medical School, Çukurova University, Adana, Turkey
d Pathology, Medical School, Çukurova University, Adana, Turkey
Accepted for publication June 13, 2003.
* Address reprint requests to Dr Topcuoglu, Department of Cardiovascular Surgery, Medical School, Çukurova University, Balcali-Adana/01330 Turkey
e-mail: sahtopcu{at}yahoo.com
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Abstract
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A case of intravenous leiomyomatosis with extention into the right ventricle is described. A tumor in the inferior vena cava was detected three years after a subtotal hysterectomy had been performed for a myomatous uterus but was misdiagnosed as a thrombus. The tumor enlarged and intruded into the right ventricle for which she underwent surgery. The correct diagnosis was made during the surgery, therefore a two-stage resection was planned. Surgical resection is the best treatment for intracardiac extention of intravenous leiomyoma. We recommend iliac venotomy to remove the ilio-caval portion of the tumor in both stages of operations.
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Introduction
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Intravenous leiomyomatosis with cardiac extension was published in 1907 from an autopsy analysis [1]. According to our literature search, at least 34 cases have been reported involving the right ventricle. Most patients are in the fifth and early sixth decades of life. These patients are sometimes asymptomatic. The occlusion of inferior vena cava leads to typical signs and symptoms of right heart failure [2]. Total obstruction of the tricuspid valve will result in sudden death. We present a case of intravenous leiomyoma, extending into the right ventricle, that was misdiagnosed as a thrombus and, as an easy approach, the ilio-caval portion of this tumor was removed via iliac venotomy.
A 58-year-old woman was admitted to our department in August 2002 for surgical excision of an inferior caval thrombus extending into the right ventricle. Her medical history included a subtotal hysterectomy for uterine myomatosis in 1997. In April 2000 she experienced several episodes of exertional dyspnea for which she was admitted to the cardiology department. An echocardiographic examination revealed a mass occupying the right atrium and a portion of this mass could be seen extending into the inferior vena cava. However, the diagnosis was intracaval thrombus at that time and the patient received low molecular weight heparin and oral anticoagulants. The patient was discharged after a control echocardiography showed no sign of thrombus following therapy and she was scheduled for evaluations every 6 months. In August 2002 her control echocardiography revealed a right ventricular thrombus and she was referred to our department (Fig 1).
Computed tomographic scan showed a mass originating from right common and internal iliac veins, which invaded the inferior vena cava (Fig 2).
Due to the risk of sudden death, the patient was immediately operated on. The operation was performed with median sternotomy. Cardiopulmonary bypass was established with ascending aortic arterial return and venous drainage through the right atrial appendage. The systemic temperature was reduced to 22°C, and total circulatory arrest was started. The right atrium was opened and, instead of a thrombus, a tumor was found extending from the right atrium through the tricuspid valve into the right ventricle. The tumor did not show attachment sites to the walls of the inferior vena cava (IVC) or the right atrium. A frozen section of the biopsy specimen taken intraoperatively was evaluated as a benign neoplasm, consistent with a smooth muscle tumor; surgical procedure was switched to a two-stage operation to enable a hysterectomy in the second stage. The tumor was removed, incompletely, after a gentle traction was applied to the tumor limb extending from the IVC into the right atrium (Fig 3A).
The postoperative period was uneventful and the patient was discharged from the hospital on the tenth day. She was readmitted in December 2002 for the second stage operation. She underwent surgery and a midline laparotomy was performed. First, the remaining uterus and the ovaries bilaterally were removed. Then incision of the right iliac vein was made. Intracaval and intrailiac tumor plugs were successfully drawn out downward through this venotomy (Fig 3B). The patient's postoperative course was uneventful. Postoperative computed tomography and echocardiography showed no intracardiac or intracaval tumors (Fig 4).
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Fig 1. Echocardiogram demonstrating a mass extending from the right atrium (RA) to the right ventricle (RV) through the tricuspid valve, which was initially thought to be a thrombus (arrows).
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Fig 3. (A) The operative specimen exhibiting an intracardiac and an intracaval component. The right ventricle component was cut (arrow); (B) right iliac and caval component.
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Comment
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Intravenous leiomyomatosis is a rare benign neoplasm, which is characterized by the easily identifiable growth of smooth muscle within the lumen of veins. The tumor most commonly enters through the lumen of the iliac vein, and grows into the inferior vena cava, sometimes reaching the right atrium, ventricle, and pulmonary artery [3–5]. Occasionally, the ovarian vein provides an alternative route to the subphrenic segment of the inferior vena cava.
Intravenous leiomyomatosis with right ventricular extension has been reported in at least 34 cases. All reported patients were women, ranging in age from 26 to 72 with a mean age of 47 years, and a history of uterine leiomyoma was observed in each case [3].
The unusual features of this case include the long delay (5 years) from the performance of subtotal hysterectomy to the presentation of caval and cardiac extension, which is often misdiagnosed as intracaval or intracardiac thrombi [5]. Although leiomyoma is histologically benign and characterized by slow growth, since intravenous leiomyomatosis can lead to complications, a total hysterectomy instead of a subtotal hysterectomy may be the appropriate approach for women near menopausal age.
Ultrasonography, echocardiography, and computed tomography were the cornerstones of our diagnosis. To prevent possible complications due to intracardiac leiomyomatosis, an urgent removal of the intracardiac lesion is vital. Complete surgical resection at the tumor is the therapy of choice for intravenous leiomyomatosis. Mandelbaum and colleagues [4] described, in 1974, the first successful resection of leiomyoma in the right heart which originated in the IVC just below the right atrium. Various surgical approaches have been used for the removal of the tumor. If the tumor is too extensive, or adheres to the cardiac and vascular structures requiring resection of the abdominopelvic and intrathoracic components, then a separate operation may be mandatory [6]. Otherwise, one-stage resection under total circulatory arrest and hypothermia can be used with success [3]. In this case we decided to do a two-stage operation because the tumor located within the inferior vena cava and right ventricle was misdiagnosed as a thrombus and the correct diagnosis was made intraoperatively. Therefore, in the first step, the intracardiac mass was removed en bloc after gentle traction was applied to the tumor limb extending from the inferior vena cava. For the removal of the intracaval tumor, a literature review showed a preference of supra and infrarenal vena cava venotomy [3, 7, 8]. However, as the leiomyoma usually does not adhere to the vessel wall, for removal of the remaining caval portion we recommend iliac venotomy. We believe this incision has these advantages over caval and especially suprarenal caval level incisions: (1) Postoperative fast recovery due to less retroperitoneal exploration; (2) Surgical complications of the iliac region are easier to cope with than the caval region; (3) Venous thrombosis due to the venotomy is more tolerable in the iliac region; (4) Better cosmetic results. (5) The advantage of being closer to the origin of the tumor, which is in the uterus.
In conclusion, surgical resection is the best treatment for intracardiac extension of intravenous leiomyoma and must be performed immediatly because of sudden death. We recommend iliac venotomy as a simple and safe approach to remove the ilio-caval portion of the tumor in intravascular leiomyomatosis one- and two-stage operations.
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References
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- Durck H. Ein kontinuierlich durch die untere. Hohlvene in das Herz vorwaschsendes Leiomyom. München Med Wochnschr 1907;54:1154–5
- Casillas J., Lobato J., Llamas P., Meseguer J. Intrathoracic extension of intravenous leiomyomatosis: a case report. J Cardiovasc Surg 1997;38:83-86.[Medline]
- Wakiyama H., Sugimoto T., Ataka K., et al. Intravenous leiomyomatosis extending into the right ventricular cavity: one-stage radical operations using cardiopulmonary bypass—a case report. Angiology 2000;51:505-509.
- Mandelbaum I., Paulletto F.J., Nasser W.K. Resection of a leiomyoma of the inferior vena cava that produced tricuspid valvular obstruction. J Thorac Cardiovasc Surg 1974;67:561-567.[Medline]
- Okamoto H., Itoh T., Morita S., Matsuura A., Yasuura K. Intravenous leiomyomatosis extending into the right ventricle: one-stage radical excision during hypothermic circulatory arrest. Thorac Cardiovasc Surg 1994;42:361-363.[Medline]
- Filsoufi F., Farivar R.S., Anderson C., Santerre D., Adams D.H. Renal vein injury complicating removal of intravenous leiomyoma. J Thorac Cardiovasc Surg 2002;123:820-822.[Free Full Text]
- Stolf N.A.G., Santos G.G., Haddad V.L.S., et al. Successful one-stage resection of the intravenous leiomyomatosis of the uterus with extension into the heart. Cardiovasc Surg 1999;7:661-664.[Medline]
- Katsumata T., Shinfeld A., Houel R., Westaby S. Pelvic leiomyoma in the right atrium. Ann Thorac Surg 1998;66:2095-2096.[Abstract/Free Full Text]
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Abstract
Introduction
