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Intravenous Leiomyomatosis with Intracardiac Extension: First Reported Case in Australia
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Brief Communication
Intravenous Leiomyomatosis with Intracardiac Extension: First Reported Case in Australia Taranpreet Singh, FRACS a,∗ , Philip M. Lamont, FRACS c , Geoffrey R. Otton, FRANZCOG b and Duncan S. Thomson, FRACS a b
a Department of Cardiothoracic Surgery, John Hunter Hospital, Newcastle, Australia Department of Obstetrics and Gynaecology, John Hunter Hospital, Newcastle, Australia c Department of Vascular Surgery, John Hunter Hospital, Newcastle, Australia
Intravenous leiomyomatosis (IVL) with cardiac extension is a rare uterine tumour. We present an unusual case of uterine leiomyoma that progressed along the inferior vena cava into the right atrium. Complete one stage removal of the tumour was performed using cardiopulmonary bypass and circulatory arrest. The literature review reveals that this is the first reported case in Australia of IVL with intracardiac extension which was successfully removed with a single stage procedure. (Heart, Lung and Circulation 2010;19:50–52) Crown Copyright © 2009 Published by Elsevier Inc. on behalf of Australasian Society of Cardiac and Thoracic Surgeons and the Cardiac Society of Australia and New Zealand. All rights reserved. Keywords. Intravenous leiomyomatosis; Leiomyoma; Leiomyomatosis; Right atrial mass; Cardiac tumour; Fibroid uterus
Introduction
I
VL is a rare benign tumour which is characterised histologically by smooth muscle cells. It is usually confined to the pelvic veins but very rarely can progress to the inferior vena cava, and even to the heart. Intracardiac leiomyomatosis (ICL) can result in life threatening symptoms and appropriate therapy is complete excision of the tumour. The case of ICL presented here depicts diagnostic and surgical difficulties that can arise from the rarity and complexity of this condition.
Case A 43-year-old woman was referred to our department with thrombosis of the inferior vena cava (IVC) and a right atrial (RA) mass. She had been experiencing right upper abdominal pain, bloating and swelling of the left leg. Computed tomography revealed RA, IVC and left common iliac veins occupied with a large filling defect and a separate tumour mass in the uterus (Fig. 1). Her past medical history included hysteroscopy, dilatation and curettage, which was reported as normal. The transabdominal and the transvaginal ultrasounds revealed three heterogeneous mass lesions in the uterus. With the diagnosis of Received 13 August 2008; received in revised form 19 October 2008; accepted 24 February 2009; available online 6 May 2009 ∗ Corresponding author at: Department of Cardiothoracic Surgery, John Hunter Hospital, Locked Bag 1, Hunter Region Mail Centre, Newcastle, NSW 2306, Australia. Tel.: +61 2 49214509; fax: +61 2 49214544. E-mail address: [email protected] (T. Singh).
uterine fibroids, conservative treatment was advised by her gynaecologist. Echocardiography revealed a large RA mass (Fig. 2) with obstructed flow into the right ventricle and what was thought to be a separate mass nearly obstructing the IVC with extension into the RA. The cardiologist’s opinion was that the RA mass was a Myxoma with a different echo texture from the IVC with thrombosis. Vascular surgical opinion was that with long standing IVC thrombosis and collateral formation, no intervention was required. So with a preoperative diagnosis of RA Myxoma and IVC thrombosis, surgery was planned for excision of the RA Myxoma. Bypass was established with aortic and venous cannulae to the superior vena cava. The RA was opened and a pump sucker was used to capture venous return from the IVC. A large tumour was found occupying most of the RA and continuing into the IVC. A frozen section reported a soft tissue tumour suggestive of smooth muscle cell origin. The vascular surgeon extended the midline incision and after mobilising bowel loops to the left side found the IVC tumour extending into the left iliac veins and to veins in the left parametriumbroad ligament. The uterus was found to be significantly enlarged. The gynaecologist performed a hysterectomy with bilateral salpingo oophorectomy. Under circulatory arrest the cardiac extent of the tumour was excised in two pieces (51 g, 60 mm × 50 mm × 45 mm and 32 g, 40 mm × 40 mm × 30 mm) and the rest of the tumour (185 g, 160 mm × 50 mm × 40 mm) was removed after opening the IVC (Fig. 3). After closure of the IVC and the RA, bypass was re-established and the patient was warmed and weaned from CPB. She made an uneventful recovery and was discharged on the tenth postoperative day. The histo-
Crown Copyright © 2009 Published by Elsevier Inc. on behalf of Australasian Society of Cardiac and Thoracic Surgeons and the Cardiac Society of Australia and New Zealand. All rights reserved.
1443-9506/04/$36.00 doi:10.1016/j.hlc.2009.02.006
Singh et al. IVL with intracardiac extension
51
staged procedure with the team effort of the gynaecologist, vascular and cardiothoracic surgeons.
Discussion
Figure 1. CT scan showing the IVC and the left common iliac veins occupied with a large filling defect and a separate tumour mass in the uterus.
Figure 2. Echocardiography showing a large RA mass.
logical examination of the uterus and intravascular tumour confirmed diagnosis of IVL. Though the mass was misdiagnosed as an RA Myxoma with a coincidental finding of IVC thrombosis, it was removed successfully as a single
IVL is usually seen in women in the fourth or fifth decade. Presentation is usually due to decreased venous return or from venous obstruction. IVL was first identified in 1896 by Birch-Hirschfeld [1]. Durk and Hormann reported the first intracardiac extension of IVL in two separate reports from autopsy specimens in 1907 [2,3]. The first report in English literature was not until 1959 by Marshall and Morris [4]. There are two main theories for this kind of tumour [5,6]. The first theory maintains there is an invasion of venous sinuses of uterine myometrium by leiomyomatosis cells of the uterus, while the second theory suggests proliferation of smooth muscle cells of uterine and pelvic veins. ICL can cause severe heart failure, pulmonary embolisation or sudden death due to incarceration into the atrio-venous orifice. Therefore urgent removal of the tumour is essential. Surgery is the treatment of choice and complete removal of tumour is mandatory because recurrences are frequent despite the benign nature of this neoplasm. Since the tumour does not grow into or attach itself to vena cava walls or cardiac chambers total excision is possible. The first successful extraction of an intracardiac portion of tumour was reported by Timmis et al. in 1980 [7]. The first total resection was reported in 1982 by Ariza et al. which was done as a two staged procedure [8]. Since that time several cases of successful and complete removal of ICL have been described [9–11]. In an extensive search by Kocica et al., till 2005 a total of 113 cases of ICL have been reported in literature [12]. Different surgical techniques have been utilised using either a single or double staged operation. The two staged operation involves removal of intrathoracic and abdominal/pelvic components in two separate operations. Recently a single staged procedure using cardiopulmonary bypass with or without circulatory arrest has been employed for total excision of the tumour. In this case we used a single staged procedure with circulatory arrest for complete removal of the tumour. As happened in our case, because of its rarity and the peculiar nature of this tumour, it is frequently misdiagnosed as intracardiac or intracaval thrombi [13]. In summary IVL with intracardiac extension is a complex disease. Accurate preoperative delineation of tumour extension is essential for the success of complete tumour excision. The cure of this complex disease emphasises the need for a planned approach by a multidisciplinary surgical team.
References
Figure 3. Picture showing the uterus mass with IVC (arrow heads) and RA (arrow) extension.
[1] Birch-Hirschfeld FV. Lehrbuch der Pathologischen Anatomie. 5th ed. Leipzig, Germany: F C W Vogel; 1896. [2] Durck H. Ueber ien Kontinvierlich durch die entere Holhlvene in das Herz vorwachsendes: fibromyom des uterus. Munchen Med Wehnschr 1907;54:1154. [3] Hormann K. Ueber einen fall von myomatosem uterustumor. Zentralbl Gynaekol 1907;31:1604–5.
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Heart, Lung and Circulation 2010;19:50–52
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Singh et al. IVL with intracardiac extension
[4] Marshall JF, Morris DS. Intravenous leiomyomatosis of the uterus with pelvis: case report. Ann Surg 1959;149:126–34. [5] Noris HJ, Parmley T. Mesenchymal tumors of the uterus. v. intravenous leimyomatosis: a clinical and pathological study of 14 cases. Cancer 1975;36:2164–78. [6] Borland DS, Wotring JW. Intravenous leiomyomatosis of the uterus and broad ligament: report of a case. Am J Clin Pathol 1964;42:182–8. [7] Timmis AD, Smallpeice C, Davies AC, Macarthur AM, Gishen P, Jackson G. Intracardiac spread of intravenous leiomyomatosis with successful surgical excision. N Engl J Med 1980;303:1043–4. [8] Ariza A, Cerra C, Hahn IS, Shaw RK, Rigney B. Intravascular leiomyomatosis of the Uterus. Conn Med 1982;46:700–3. [9] Harris LM, Karakousis CP. Intravenous leiomyomatosis with cardiac extension: tumor thrombectomy through an abdominal approach. J Vasc Surg 2000;31:1046–51.
Heart, Lung and Circulation 2010;19:50–52
[10] Nam MS, Jeon MJ, Kim YE, Kim JW, Park KH, Hong YS. Pelvic leiomyomatosis with intracaval and intracardiac extension: a case report and review of the literature. Gynecol Oncol 2003;89:175–80. [11] Lam PM, Lo KWK, Yu MY, Wong WS, Lau JYW, Arifi AA, Cheung TH. Intravenous leiomyomatosis with atypical histologic features: a case report. Int J Gynecol Cancer 2003;13:83–7. [12] Kocica MJ, Vranes MR, Kostic D, Kovacevic-Kostic N, Lackovic V, Bozic-Mihajlovic V, Velinovic MM, Mikic ADj, DimitrijevicKalezic N. Intravenous leiomyomatosis with extension to the heart: rare or underestimated? J Thorac Cardiovasc Surg 2005;130(6):1724–6. [13] Farfel Z, Shechter M, Vered Z, Rath S, Goor D, Gafni J. Review of echocardiographically diagnosed right heart entrapment of pulmonary emboli-in-transit with emphasis on management. Am Heart J 1987;113:171–8.
Brief Communication
Intravenous Leiomyomatosis with Intracardiac Extension: First Reported Case in Australia Taranpreet Singh, FRACS a,∗ , Philip M. Lamont, FRACS c , Geoffrey R. Otton, FRANZCOG b and Duncan S. Thomson, FRACS a b
a Department of Cardiothoracic Surgery, John Hunter Hospital, Newcastle, Australia Department of Obstetrics and Gynaecology, John Hunter Hospital, Newcastle, Australia c Department of Vascular Surgery, John Hunter Hospital, Newcastle, Australia
Intravenous leiomyomatosis (IVL) with cardiac extension is a rare uterine tumour. We present an unusual case of uterine leiomyoma that progressed along the inferior vena cava into the right atrium. Complete one stage removal of the tumour was performed using cardiopulmonary bypass and circulatory arrest. The literature review reveals that this is the first reported case in Australia of IVL with intracardiac extension which was successfully removed with a single stage procedure. (Heart, Lung and Circulation 2010;19:50–52) Crown Copyright © 2009 Published by Elsevier Inc. on behalf of Australasian Society of Cardiac and Thoracic Surgeons and the Cardiac Society of Australia and New Zealand. All rights reserved. Keywords. Intravenous leiomyomatosis; Leiomyoma; Leiomyomatosis; Right atrial mass; Cardiac tumour; Fibroid uterus
Introduction
I
VL is a rare benign tumour which is characterised histologically by smooth muscle cells. It is usually confined to the pelvic veins but very rarely can progress to the inferior vena cava, and even to the heart. Intracardiac leiomyomatosis (ICL) can result in life threatening symptoms and appropriate therapy is complete excision of the tumour. The case of ICL presented here depicts diagnostic and surgical difficulties that can arise from the rarity and complexity of this condition.
Case A 43-year-old woman was referred to our department with thrombosis of the inferior vena cava (IVC) and a right atrial (RA) mass. She had been experiencing right upper abdominal pain, bloating and swelling of the left leg. Computed tomography revealed RA, IVC and left common iliac veins occupied with a large filling defect and a separate tumour mass in the uterus (Fig. 1). Her past medical history included hysteroscopy, dilatation and curettage, which was reported as normal. The transabdominal and the transvaginal ultrasounds revealed three heterogeneous mass lesions in the uterus. With the diagnosis of Received 13 August 2008; received in revised form 19 October 2008; accepted 24 February 2009; available online 6 May 2009 ∗ Corresponding author at: Department of Cardiothoracic Surgery, John Hunter Hospital, Locked Bag 1, Hunter Region Mail Centre, Newcastle, NSW 2306, Australia. Tel.: +61 2 49214509; fax: +61 2 49214544. E-mail address: [email protected] (T. Singh).
uterine fibroids, conservative treatment was advised by her gynaecologist. Echocardiography revealed a large RA mass (Fig. 2) with obstructed flow into the right ventricle and what was thought to be a separate mass nearly obstructing the IVC with extension into the RA. The cardiologist’s opinion was that the RA mass was a Myxoma with a different echo texture from the IVC with thrombosis. Vascular surgical opinion was that with long standing IVC thrombosis and collateral formation, no intervention was required. So with a preoperative diagnosis of RA Myxoma and IVC thrombosis, surgery was planned for excision of the RA Myxoma. Bypass was established with aortic and venous cannulae to the superior vena cava. The RA was opened and a pump sucker was used to capture venous return from the IVC. A large tumour was found occupying most of the RA and continuing into the IVC. A frozen section reported a soft tissue tumour suggestive of smooth muscle cell origin. The vascular surgeon extended the midline incision and after mobilising bowel loops to the left side found the IVC tumour extending into the left iliac veins and to veins in the left parametriumbroad ligament. The uterus was found to be significantly enlarged. The gynaecologist performed a hysterectomy with bilateral salpingo oophorectomy. Under circulatory arrest the cardiac extent of the tumour was excised in two pieces (51 g, 60 mm × 50 mm × 45 mm and 32 g, 40 mm × 40 mm × 30 mm) and the rest of the tumour (185 g, 160 mm × 50 mm × 40 mm) was removed after opening the IVC (Fig. 3). After closure of the IVC and the RA, bypass was re-established and the patient was warmed and weaned from CPB. She made an uneventful recovery and was discharged on the tenth postoperative day. The histo-
Crown Copyright © 2009 Published by Elsevier Inc. on behalf of Australasian Society of Cardiac and Thoracic Surgeons and the Cardiac Society of Australia and New Zealand. All rights reserved.
1443-9506/04/$36.00 doi:10.1016/j.hlc.2009.02.006
Singh et al. IVL with intracardiac extension
51
staged procedure with the team effort of the gynaecologist, vascular and cardiothoracic surgeons.
Discussion
Figure 1. CT scan showing the IVC and the left common iliac veins occupied with a large filling defect and a separate tumour mass in the uterus.
Figure 2. Echocardiography showing a large RA mass.
logical examination of the uterus and intravascular tumour confirmed diagnosis of IVL. Though the mass was misdiagnosed as an RA Myxoma with a coincidental finding of IVC thrombosis, it was removed successfully as a single
IVL is usually seen in women in the fourth or fifth decade. Presentation is usually due to decreased venous return or from venous obstruction. IVL was first identified in 1896 by Birch-Hirschfeld [1]. Durk and Hormann reported the first intracardiac extension of IVL in two separate reports from autopsy specimens in 1907 [2,3]. The first report in English literature was not until 1959 by Marshall and Morris [4]. There are two main theories for this kind of tumour [5,6]. The first theory maintains there is an invasion of venous sinuses of uterine myometrium by leiomyomatosis cells of the uterus, while the second theory suggests proliferation of smooth muscle cells of uterine and pelvic veins. ICL can cause severe heart failure, pulmonary embolisation or sudden death due to incarceration into the atrio-venous orifice. Therefore urgent removal of the tumour is essential. Surgery is the treatment of choice and complete removal of tumour is mandatory because recurrences are frequent despite the benign nature of this neoplasm. Since the tumour does not grow into or attach itself to vena cava walls or cardiac chambers total excision is possible. The first successful extraction of an intracardiac portion of tumour was reported by Timmis et al. in 1980 [7]. The first total resection was reported in 1982 by Ariza et al. which was done as a two staged procedure [8]. Since that time several cases of successful and complete removal of ICL have been described [9–11]. In an extensive search by Kocica et al., till 2005 a total of 113 cases of ICL have been reported in literature [12]. Different surgical techniques have been utilised using either a single or double staged operation. The two staged operation involves removal of intrathoracic and abdominal/pelvic components in two separate operations. Recently a single staged procedure using cardiopulmonary bypass with or without circulatory arrest has been employed for total excision of the tumour. In this case we used a single staged procedure with circulatory arrest for complete removal of the tumour. As happened in our case, because of its rarity and the peculiar nature of this tumour, it is frequently misdiagnosed as intracardiac or intracaval thrombi [13]. In summary IVL with intracardiac extension is a complex disease. Accurate preoperative delineation of tumour extension is essential for the success of complete tumour excision. The cure of this complex disease emphasises the need for a planned approach by a multidisciplinary surgical team.
References
Figure 3. Picture showing the uterus mass with IVC (arrow heads) and RA (arrow) extension.
[1] Birch-Hirschfeld FV. Lehrbuch der Pathologischen Anatomie. 5th ed. Leipzig, Germany: F C W Vogel; 1896. [2] Durck H. Ueber ien Kontinvierlich durch die entere Holhlvene in das Herz vorwachsendes: fibromyom des uterus. Munchen Med Wehnschr 1907;54:1154. [3] Hormann K. Ueber einen fall von myomatosem uterustumor. Zentralbl Gynaekol 1907;31:1604–5.
BRIEF COMMUNICATION
Heart, Lung and Circulation 2010;19:50–52
BRIEF COMMUNICATION
52
Singh et al. IVL with intracardiac extension
[4] Marshall JF, Morris DS. Intravenous leiomyomatosis of the uterus with pelvis: case report. Ann Surg 1959;149:126–34. [5] Noris HJ, Parmley T. Mesenchymal tumors of the uterus. v. intravenous leimyomatosis: a clinical and pathological study of 14 cases. Cancer 1975;36:2164–78. [6] Borland DS, Wotring JW. Intravenous leiomyomatosis of the uterus and broad ligament: report of a case. Am J Clin Pathol 1964;42:182–8. [7] Timmis AD, Smallpeice C, Davies AC, Macarthur AM, Gishen P, Jackson G. Intracardiac spread of intravenous leiomyomatosis with successful surgical excision. N Engl J Med 1980;303:1043–4. [8] Ariza A, Cerra C, Hahn IS, Shaw RK, Rigney B. Intravascular leiomyomatosis of the Uterus. Conn Med 1982;46:700–3. [9] Harris LM, Karakousis CP. Intravenous leiomyomatosis with cardiac extension: tumor thrombectomy through an abdominal approach. J Vasc Surg 2000;31:1046–51.
Heart, Lung and Circulation 2010;19:50–52
[10] Nam MS, Jeon MJ, Kim YE, Kim JW, Park KH, Hong YS. Pelvic leiomyomatosis with intracaval and intracardiac extension: a case report and review of the literature. Gynecol Oncol 2003;89:175–80. [11] Lam PM, Lo KWK, Yu MY, Wong WS, Lau JYW, Arifi AA, Cheung TH. Intravenous leiomyomatosis with atypical histologic features: a case report. Int J Gynecol Cancer 2003;13:83–7. [12] Kocica MJ, Vranes MR, Kostic D, Kovacevic-Kostic N, Lackovic V, Bozic-Mihajlovic V, Velinovic MM, Mikic ADj, DimitrijevicKalezic N. Intravenous leiomyomatosis with extension to the heart: rare or underestimated? J Thorac Cardiovasc Surg 2005;130(6):1724–6. [13] Farfel Z, Shechter M, Vered Z, Rath S, Goor D, Gafni J. Review of echocardiographically diagnosed right heart entrapment of pulmonary emboli-in-transit with emphasis on management. Am Heart J 1987;113:171–8.