Intracardiac Extension of Intravenous Leiomyomatosis

Intravenous leiomyomatosis with intracardiac extension is a rare condition characterized by extensive growth of a benign uterine mass that extends into the venous system through uterine channels and then into the cardiac chambers. A variety of presentations exist; cure relies on complete surgical resection. Extensive abdominal dissection, cardiopulmonary bypass (with or without circulatory arrest), and removal of the intracaval component are required. However, because of the rarity and variety of presentation, exact preferred management has not been well defined. A specific case, followed by a comprehensive literature review, helps delineate the specific decision making necessary for mass removal. Intravenous leiomyomatosis with intracardiac extension is a rare condition characterized by extensive growth of a benign uterine mass that extends into the venous system through uterine channels and then into the cardiac chambers. A variety of presentations exist; cure relies on complete surgical resection. Extensive abdominal dissection, cardiopulmonary bypass (with or without circulatory arrest), and removal of the intracaval component are required. However, because of the rarity and variety of presentation, exact preferred management has not been well defined. A specific case, followed by a comprehensive literature review, helps delineate the specific decision making necessary for mass removal. Intravenous leiomyomatosis is a phenomenon occurring in parous, premenopausal women with a history of uterine leiomyomata; intracardiac extension is extremely rare [1Cooper M. Guillem J. Dalton J. et al.Recurrent intravenous leiomyomatosis with cardiac extension.Ann Thorac Surg. 1992; 53: 139-141Abstract Full Text PDF PubMed Scopus (53) Google Scholar, 2Steinmetz O. Bedard P. Prefontaine M.E. Bourke M. Barber G.G. Uterine tumor in the heart: intravenous leiomyomatosis.Surgery. 1996; 119: 226-229Abstract Full Text PDF PubMed Scopus (22) Google Scholar, 3Roques F. Sanchez Bucher B. Lariviere J. Role of pre-operative assessment in the surgical management of leiomyoma extended to the right heart chambers: a compendium of information from isolated reports.Eur J Cardiothorac Surg. 2001; 19: 522-524Crossref PubMed Scopus (28) Google Scholar, 4Gan H. Zang J. Zhou Q. Kong Q. Zhao H. Bo P. Surgical treatment of intracardiac leiomyomatosis.J Thorac Cardiovasc Surg. 2011; 142: 823-828Abstract Full Text Full Text PDF PubMed Scopus (12) Google Scholar, 5Lam P. Lo K. Yu M. et al.Intravenous leiomyomatosis: two cases with different routes of tumor extension.J Vasc Surg. 2004; 39: 465-469Abstract Full Text Full Text PDF PubMed Scopus (103) Google Scholar, 6Kocica M.J. Vranes M.R. Kostic D. et al.Intravenous leiomyomatosis with extension to the heart: rare or underestimated?.J Thorac Cardiovasc Surg. 2005; 130: 1724-1726Abstract Full Text Full Text PDF PubMed Scopus (57) Google Scholar, 7Singh T. Lamont P. Otton G. Thomson D.S. Intravenous leiomyomatosis with intracardiac extension: first reported case in Australia.Heart Lung Circ. 2010; 19: 50-52Abstract Full Text Full Text PDF PubMed Scopus (18) Google Scholar, 8Lou Y. Shi X. Song Z. Intravenous leiomyomatosis of the uterus with extension to the right heart.Cardiovasc Ultrasound. 2011; 9: 25-29Crossref PubMed Scopus (31) Google Scholar]. This tumor can lead to profound physiologic disturbances resulting in advanced heart failure and sudden death [2Steinmetz O. Bedard P. Prefontaine M.E. Bourke M. Barber G.G. Uterine tumor in the heart: intravenous leiomyomatosis.Surgery. 1996; 119: 226-229Abstract Full Text PDF PubMed Scopus (22) Google Scholar]. Patient presentation may be extremely variable, and the ideal modalities of workup have not been described [2Steinmetz O. Bedard P. Prefontaine M.E. Bourke M. Barber G.G. Uterine tumor in the heart: intravenous leiomyomatosis.Surgery. 1996; 119: 226-229Abstract Full Text PDF PubMed Scopus (22) Google Scholar, 3Roques F. Sanchez Bucher B. Lariviere J. Role of pre-operative assessment in the surgical management of leiomyoma extended to the right heart chambers: a compendium of information from isolated reports.Eur J Cardiothorac Surg. 2001; 19: 522-524Crossref PubMed Scopus (28) Google Scholar]. There is no consensus for the optimal approach for resection of this tumor, and the procedure chosen may vary widely among surgeons. We report a patient presenting with advanced disease and offer a detailed review of the appropriate workup and operative technique to ensure a complete and long-term cure. The patient is a 53-year-old gravida 0, para 0, woman with a history of partial hysterectomy for uterine fibroids. Her presentation was significant for intermittent vaginal bleeding, abdominal pain, mild hypotension with tachycardia, severe lower extremity edema, and a large, firm, nontender mass in the lower abdomen. Transesophageal echocardiography demonstrated a large, well-defined mass extending from the vena cava into the right atrium without a clear site of attachment within the heart. Computed tomography (CT) angiography and magnetic resonance imaging of the abdomen and pelvis demonstrated a large, lobulated, and heterogeneous uterus with a tumor thrombus extending from the right internal iliac vein into the inferior vena cava and subsequently into the right ventricle (Fig 1). Resection was planned by multidisciplinary approach. A total abdominal hysterectomy and bilateral salpingo-oophorectomy was performed through a midline laparotomy. The tumor was transected at the level of its invasion into the internal iliac vein to facilitate the removal of the uterus. A median sternotomy was then performed and cardiopulmonary bypass (CPB) initiated with arterial perfusion through the ascending aorta and venous return through the superior vena cava and left femoral vein. The right atrium was opened, and the mass was extracted through the tricuspid valve. The site of the distal attachment in the right common iliac vein was released, facilitating removal of the tumor through the right atriotomy (Fig 2). The patient was then weaned from CPB without difficulty. The uterus measured 23 cm in largest diameter and weighed 1.9 kg. The intravenous tumor measured 35 cm and was reported as a benign smooth muscle neoplasm, consistent with intravenous leiomyomatosis (Fig 3). The patient underwent screening CT without evidence of tumor recurrence at 36 months. Intravenous leiomyomatosis most commonly occurs in premenopausal women and must be considered when evaluating an intracardiac mass in the right atrium that extends from the inferior vena cava [1Cooper M. Guillem J. Dalton J. et al.Recurrent intravenous leiomyomatosis with cardiac extension.Ann Thorac Surg. 1992; 53: 139-141Abstract Full Text PDF PubMed Scopus (53) Google Scholar, 2Steinmetz O. Bedard P. Prefontaine M.E. Bourke M. Barber G.G. Uterine tumor in the heart: intravenous leiomyomatosis.Surgery. 1996; 119: 226-229Abstract Full Text PDF PubMed Scopus (22) Google Scholar, 3Roques F. Sanchez Bucher B. Lariviere J. Role of pre-operative assessment in the surgical management of leiomyoma extended to the right heart chambers: a compendium of information from isolated reports.Eur J Cardiothorac Surg. 2001; 19: 522-524Crossref PubMed Scopus (28) Google Scholar, 4Gan H. Zang J. Zhou Q. Kong Q. Zhao H. Bo P. Surgical treatment of intracardiac leiomyomatosis.J Thorac Cardiovasc Surg. 2011; 142: 823-828Abstract Full Text Full Text PDF PubMed Scopus (12) Google Scholar, 5Lam P. Lo K. Yu M. et al.Intravenous leiomyomatosis: two cases with different routes of tumor extension.J Vasc Surg. 2004; 39: 465-469Abstract Full Text Full Text PDF PubMed Scopus (103) Google Scholar, 6Kocica M.J. Vranes M.R. Kostic D. et al.Intravenous leiomyomatosis with extension to the heart: rare or underestimated?.J Thorac Cardiovasc Surg. 2005; 130: 1724-1726Abstract Full Text Full Text PDF PubMed Scopus (57) Google Scholar, 7Singh T. Lamont P. Otton G. Thomson D.S. Intravenous leiomyomatosis with intracardiac extension: first reported case in Australia.Heart Lung Circ. 2010; 19: 50-52Abstract Full Text Full Text PDF PubMed Scopus (18) Google Scholar, 8Lou Y. Shi X. Song Z. Intravenous leiomyomatosis of the uterus with extension to the right heart.Cardiovasc Ultrasound. 2011; 9: 25-29Crossref PubMed Scopus (31) Google Scholar]. This process was first described at autopsy at the turn of the last century [2Steinmetz O. Bedard P. Prefontaine M.E. Bourke M. Barber G.G. Uterine tumor in the heart: intravenous leiomyomatosis.Surgery. 1996; 119: 226-229Abstract Full Text PDF PubMed Scopus (22) Google Scholar]. Patients may present with abdominal pain, isolated syncopal episodes, pelvic mass, or a combination of these [2Steinmetz O. Bedard P. Prefontaine M.E. Bourke M. Barber G.G. Uterine tumor in the heart: intravenous leiomyomatosis.Surgery. 1996; 119: 226-229Abstract Full Text PDF PubMed Scopus (22) Google Scholar, 3Roques F. Sanchez Bucher B. Lariviere J. Role of pre-operative assessment in the surgical management of leiomyoma extended to the right heart chambers: a compendium of information from isolated reports.Eur J Cardiothorac Surg. 2001; 19: 522-524Crossref PubMed Scopus (28) Google Scholar, 4Gan H. Zang J. Zhou Q. Kong Q. Zhao H. Bo P. Surgical treatment of intracardiac leiomyomatosis.J Thorac Cardiovasc Surg. 2011; 142: 823-828Abstract Full Text Full Text PDF PubMed Scopus (12) Google Scholar, 5Lam P. Lo K. Yu M. et al.Intravenous leiomyomatosis: two cases with different routes of tumor extension.J Vasc Surg. 2004; 39: 465-469Abstract Full Text Full Text PDF PubMed Scopus (103) Google Scholar, 6Kocica M.J. Vranes M.R. Kostic D. et al.Intravenous leiomyomatosis with extension to the heart: rare or underestimated?.J Thorac Cardiovasc Surg. 2005; 130: 1724-1726Abstract Full Text Full Text PDF PubMed Scopus (57) Google Scholar, 7Singh T. Lamont P. Otton G. Thomson D.S. Intravenous leiomyomatosis with intracardiac extension: first reported case in Australia.Heart Lung Circ. 2010; 19: 50-52Abstract Full Text Full Text PDF PubMed Scopus (18) Google Scholar]. The time of diagnosis of uterine leiomyomata to time of diagnosis of intravenous leiomyomatosis is extremely variable, with some presenting as long as 30 years after total abdominal hysterectomy. With evidence of cardiovascular symptoms, echocardiography is the preliminary test of choice [1Cooper M. Guillem J. Dalton J. et al.Recurrent intravenous leiomyomatosis with cardiac extension.Ann Thorac Surg. 1992; 53: 139-141Abstract Full Text PDF PubMed Scopus (53) Google Scholar]. Transthoracic echocardiography may yield important information but is not sufficient to make the diagnosis. Misdiagnosis with primary cardiac masses or inferior vena cava thrombosis may result if transthoracic echocardiography alone is used [3Roques F. Sanchez Bucher B. Lariviere J. Role of pre-operative assessment in the surgical management of leiomyoma extended to the right heart chambers: a compendium of information from isolated reports.Eur J Cardiothorac Surg. 2001; 19: 522-524Crossref PubMed Scopus (28) Google Scholar, 7Singh T. Lamont P. Otton G. Thomson D.S. Intravenous leiomyomatosis with intracardiac extension: first reported case in Australia.Heart Lung Circ. 2010; 19: 50-52Abstract Full Text Full Text PDF PubMed Scopus (18) Google Scholar]. CT and magnetic resonance imaging are used to better characterize the complex abdominal mass. Both can demonstrate the origin of the tumor and its site of venous invasion. CT is more rapid and less expensive, but magnetic resonance imaging is better able to identify differences in soft tissue composition, which may facilitate identification of specific points of attachment of the intravenous component. The treatment of intravenous leiomyomatosis has two goals: (1) to remove the intracardiac burden on right heart and (2) prevent recurrence. The first complete excision of intracardiac extension of uterine leiomyomata was described in 1982 by Ariza and colleagues [9Ariza A. Cerra C. Hahn I.S. Shaw R.K. Rigney B. Intravascular leiomyomatosis of the uterus. A case report.Conn Med. 1982; 46: 700-703PubMed Google Scholar] through a two-stage approach. Since then, surgical approaches have varied from two-stage [5Lam P. Lo K. Yu M. et al.Intravenous leiomyomatosis: two cases with different routes of tumor extension.J Vasc Surg. 2004; 39: 465-469Abstract Full Text Full Text PDF PubMed Scopus (103) Google Scholar, 6Kocica M.J. Vranes M.R. Kostic D. et al.Intravenous leiomyomatosis with extension to the heart: rare or underestimated?.J Thorac Cardiovasc Surg. 2005; 130: 1724-1726Abstract Full Text Full Text PDF PubMed Scopus (57) Google Scholar] to single procedures, providing complete intracardiac and pelvic mass excision at one setting [1Cooper M. Guillem J. Dalton J. et al.Recurrent intravenous leiomyomatosis with cardiac extension.Ann Thorac Surg. 1992; 53: 139-141Abstract Full Text PDF PubMed Scopus (53) Google Scholar, 2Steinmetz O. Bedard P. Prefontaine M.E. Bourke M. Barber G.G. Uterine tumor in the heart: intravenous leiomyomatosis.Surgery. 1996; 119: 226-229Abstract Full Text PDF PubMed Scopus (22) Google Scholar, 3Roques F. Sanchez Bucher B. Lariviere J. Role of pre-operative assessment in the surgical management of leiomyoma extended to the right heart chambers: a compendium of information from isolated reports.Eur J Cardiothorac Surg. 2001; 19: 522-524Crossref PubMed Scopus (28) Google Scholar, 7Singh T. Lamont P. Otton G. Thomson D.S. Intravenous leiomyomatosis with intracardiac extension: first reported case in Australia.Heart Lung Circ. 2010; 19: 50-52Abstract Full Text Full Text PDF PubMed Scopus (18) Google Scholar, 8Lou Y. Shi X. Song Z. Intravenous leiomyomatosis of the uterus with extension to the right heart.Cardiovasc Ultrasound. 2011; 9: 25-29Crossref PubMed Scopus (31) Google Scholar]. There has been some work seeking to identify specific tumor morphologies that can guide surgical planning [4Gan H. Zang J. Zhou Q. Kong Q. Zhao H. Bo P. Surgical treatment of intracardiac leiomyomatosis.J Thorac Cardiovasc Surg. 2011; 142: 823-828Abstract Full Text Full Text PDF PubMed Scopus (12) Google Scholar]. This demonstrates promise but has yet to be validated, and the ideal procedure is still guided by patient-specific disease. A single-stage operative approach using a multidisciplinary team has been adopted as a safe and effective technique for complete resection in patients who are good surgical candidates. The decision to stage procedures is determined by the patient's stability, physiologic reserve, and the complexity of the pelvic component. Resection of the intracardiac tumor burden alone has been performed in patients unable to safely undergo extensive pelvic dissection; this method requires frequent follow-up with serial imaging to evaluate for residual tumor growth. There are a number of modalities to consider to support the circulation and provide a bloodless field for resection. Deep hypothermic circulatory arrest is a well-documented means of gaining good visualization of the right atrium for removal of the tumor and also providing a bloodless field for removal of the intravascular tumor in the abdominal inferior vena cava. Single-stage deep hypothermic circulatory arrest has been highly advocated for cavoatrial tumors and tumor thrombus. CPB without circulatory arrest has also been described and used with similar outcomes [3Roques F. Sanchez Bucher B. Lariviere J. Role of pre-operative assessment in the surgical management of leiomyoma extended to the right heart chambers: a compendium of information from isolated reports.Eur J Cardiothorac Surg. 2001; 19: 522-524Crossref PubMed Scopus (28) Google Scholar, 6Kocica M.J. Vranes M.R. Kostic D. et al.Intravenous leiomyomatosis with extension to the heart: rare or underestimated?.J Thorac Cardiovasc Surg. 2005; 130: 1724-1726Abstract Full Text Full Text PDF PubMed Scopus (57) Google Scholar, 7Singh T. Lamont P. Otton G. Thomson D.S. Intravenous leiomyomatosis with intracardiac extension: first reported case in Australia.Heart Lung Circ. 2010; 19: 50-52Abstract Full Text Full Text PDF PubMed Scopus (18) Google Scholar]. CPB with moderate hypothermic arrest and cerebral perfusion help to avoid complications such as organ dysfunction, neurologic deficits, and the severe coagulopathy that can accompany deep hypothermic circulatory arrest. Cardioplegic cardiac arrest is another option during surgical resection. This provides a motionless surgical field that may be useful for tumor adherence or subsequent valve repair. In many cases, the right atrial tumor extension may be removed on bypass safely from a beating heart [3Roques F. Sanchez Bucher B. Lariviere J. Role of pre-operative assessment in the surgical management of leiomyoma extended to the right heart chambers: a compendium of information from isolated reports.Eur J Cardiothorac Surg. 2001; 19: 522-524Crossref PubMed Scopus (28) Google Scholar]. Definitive recommendations have not been created for postoperative follow-up. Some have proposed an initial period of visits at 3, 6, and 9 months. Follow-up care must be closely monitored because intravenous leiomyomatosis has a potentially high recurrence rate of up to 30% [2Steinmetz O. Bedard P. Prefontaine M.E. Bourke M. Barber G.G. Uterine tumor in the heart: intravenous leiomyomatosis.Surgery. 1996; 119: 226-229Abstract Full Text PDF PubMed Scopus (22) Google Scholar, 4Gan H. Zang J. Zhou Q. Kong Q. Zhao H. Bo P. Surgical treatment of intracardiac leiomyomatosis.J Thorac Cardiovasc Surg. 2011; 142: 823-828Abstract Full Text Full Text PDF PubMed Scopus (12) Google Scholar]. Ultrasonography and/or CT are used to screen for recurrence. Owing to the estrogen-dependent growth of leiomyomata, adjuvant therapy with tamoxifen has been proposed; however, it has been incompletely studied, and its efficacy is controversial. In conclusion, leiomyomatosis with intracardiac extension may present with heart failure or isolated abdominal complaints. When a premenopausal woman with a history of uterine leiomyomata presents with a finding of an intracardiac mass, further evaluation should be conducted to identify the source of the mass. A single-stage approach using CPB is a safe and effective method of tumor extraction in selected patients.