Arch Gynecol Obstet DOI 10.1007/s00404-014-3278-5
Case Report
Intravenous leiomyomatosis with intracardiac involvement Meng Xia · Junxiu Liu · Xianhong Xiang · Ming Xu · Mian He
Received: 15 March 2014 / Accepted: 28 April 2014 © Springer-Verlag Berlin Heidelberg 2014
Abstract Intravenous leiomyomatosis with intracardiac involvement is rare. This is a case report of a 52-year-old female with intravenous leiomyomatosis with intracardiac involvement. She was successfully treated with myomatectomy (left renal vein and inferior vena cava), hysterectomy, and bilateral salpingo-oophorectomy under the cardiopulmonary bypass. Keywords Intravenous leiomyomatosis · Intracardiac involvement
Introduction Intravenous leiomyomatosis is rare. Since the first description by Birch in 1896 [1], about 200 cases of intravenous leiomyomatosis have been reported in the literatures. Among these cases, about 10–30 % involve inferior vena cava, lung and even the cardiac cavity [2]. The first case of intravenous leiomyomatosis with intracardiac involvement was also described by Birch in 1906 [1]. So far, about 78 cases have been reported in the literatures.
M. Xia · J. Liu · M. He (*) Department of Gynecology and Obstetrics, The First Affiliated Hospital of Sun YAT-sen University, Guangzhou 510080, China e-mail: [email protected] X. Xiang Department of Interventional Radiology, The First Affiliated Hospital of Sun YAT-sen University, Guangzhou 510080, China M. Xu Department of Ultrasound, The First Affiliated Hospital of Sun YAT-sen University, Guangzhou 510080, China
Case report A 63-year-old female presented with a year of recurrent chest tightness, syncope and abdominal distention. A month prior to admitting to hospital, she received duplex ultrasound examination. It demonstrated the deep venous thrombosis of left lower extremity involving left renal vein and inferior vena cava. She denied any other symptoms, history of surgery, or vascular disease. She received hormone therapy for abnormal uterine bleeding in 2007 and has 2-year history of uterine myoma. On general physical exam, there was no abnormal founding. Laboratory values were in normal range including blood routine, liver and renal functions. A general PET-CT scan revealed multiple hypo dense lesions in the uterus body, which had irregular shape, unclear border. The biggest lesion was located in the left side of uterine body, and the range was 4.0 × 2.0 × 3.2 cm. Lesions involved the left adnexal and the broad ligament of the uterus. From the PET-CT images, it was found that left ovarian vein, left renal vein and inferior vena cava were augmented and lumen showed low dense images. It was inferred that the uterine leiomyomatosis extended to inferior vena cava through the left ovarian vein and left renal vein, and reached right atrium. Plain, contrast-enhanced and three-dimensional reconstruction spiral CT scans showed the multiple abnormal density lesions of uterus, from which we concluded uterine leiomyomatosis spread to inferior vena cava through the left ovarian vein and left renal vein, and reached right atrium (Fig. 1). Gynecological ultrasound showed multiple small uterine fibroids. Oval fluid dark space was found in the left adnexal, which was 35 × 26 mm. The patient was diagnosed as intravenous leiomyomatosis with intracardiac involvement. After multidisciplinary
13
Arch Gynecol Obstet
Fig. 1 Imaging examination before operation. a The PET-CT scan: the multiple abnormal density lesions of uterus, and the lesions extended to inferior vena cava through the left ovarian vein and left renal vein. The lesions had not increased metabolic abnormalities. b Contrast-enhanced CT scan: the left ovarian vein, left renal vein, inferior vena cava to atrium had filling defect
discussion, it was decided that operation was the best treatment. The patient underwent myomatectomy (left renal vein and inferior vena cava), hysterectomy, and bilateral salpingo-oophorectomy under the cardiopulmonary bypass. The tumor originated from uterine was cord-like, with medium texture and pale color. It floated in the vessels and spread from the uterine and ovarian veins to right atrium (Fig. 2). The tumor was resected totally with no visible residual. Successful operation was performed. The patient recovered well after operation and received regular followup. No episode of tumor recurrence was observed during the follow-up (Fig. 3).
Discussion The cause of intravenous leiomyomatosis is still unknown. There are two kinds of etiological theories: leiomyomatosis growing from vessel wall and leiomyomatosis growing into cardiac cavity along main vessels [3, 4]. In our case, the tumor spread from uterine vessel to cardiac cavity and was not adherently tight with the vessel wall. It seemed that our case supported the theory of leiomyomatosis growing into cardiac cavity along main vessels. Kokawa et al. [5] reported a postmenopausal patient with high levels of estradiol and estrogen receptor diagnosed intravenous leiomyomatosis. It indicated that high levels of estradiol and estrogen receptor were related to the growth and spreading of intravenous leiomyomatosis. In
13
our case, the patient received hormonal therapy for abnormal uterine bleeding with the test of estradiol 1,129 pg/ml. Dal et al. [6] found that there was chromosome abnormality of translocation between 12q15 and 14q24, although there was no similar abnormality in patients with uterine myoma. It indicated that the chromosome abnormality was also related to intravenous leiomyomatosis. Early diagnosis is difficult, because patients may be asymptomatic despite extensive intravenous extension or there are some mild, non-specific symptoms including dyspnea on exertion, shortness of breath, orthopnea, pleuritic chest pain, and syncopal episodes [7]. Both exact examinations and awareness of the disease are needed. Abdominal ultrasonography and a CT scan are usually used. Despite this, there are still a few patients misdiagnosed as tumor thrombosis and accepted anticoagulation therapy in earlier time [7, 8]. PET-CT is helpful for the diagnosis. 18F-FDGPET-CT scans can be used to differentiate between benign and malignant diseases such as tumor thrombosis, as the malignant disease has increased uptake of 18-FDG, whereas the benign exhibits normal biodistribution of the agent [9]. Successful therapy relies on surgical resection. In previous displays, complete surgical excision is often precluded by involvement of the tumor in the deep tributaries of the hypogastric vessels [10]. However, isolation of residual tumor appears equally important to prevent recurrences that can necessitate further surgery or lead to death. Recurrent disease is common and can occur up to 15 years after surgery [11]. Redevelopment can also occur quite rapidly.
Arch Gynecol Obstet Fig. 2 Resected tissues. a Uterus, bilateral adnexa and tumor embolus in left ovarian rein, b tumor embolus in inferior vena cava; pathological findings (×200). c Hematoxylin and eosin (HE) staining, d–h immunohistochemical (IHC) staining, vimentin (+), Actin (+), S-100 (+), endothelial cells CD31 (+), HMB45 (−), respectively
As these lesions are at great risk to recur, close long-term follow-up is essential [12]. In order to reduce the risk of future recurrence, most patients underwent hysterectomy, bilateral salpingo-oophorectomy. Another reason for undergoing the radical operation is the elder age. Age of onset for these patients concentrated in the 40–63 years of age and most of them have no fertility requirements [7, 13, 14]. However, patients may present with intravenous leiomyomatosis with intracardiac involvement from 40 years old or even younger. There was one case reported during pregnancy. Marom et al. [15]
reported a case in which the diagnosis was made at 8 weeks of gestation, followed by delivery by Cesarean section at 36 weeks. A two-stage operation was performed after delivery. But until now, there are no studies focusing on the issue of uterine preservation for the purpose of future fertility. Hormonal therapy is also used in the past decade. The use of postoperative antiestrogens, such as tamoxifen, GnRHa, has been considered, but their efficacy has yet to be established [16–19]. Long-term efficacy comparing choices in postoperative treatment is lacking but presents an area of interest for future investigations.
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Arch Gynecol Obstet
Fig. 3 The CT scan before and after operation. a, b The CT scan before operation and c, d the CT scan on the 64th day after operation showed the left ovarian vein disappeared and the recanalization of left renal vein and Inferior vena cava
In conclusion, intravenous leiomyomatosis with intracardiac involvement is a rare disease that there are no specific presentations. Operation is the best treatment and total resection can prevent recurrence effectively. Hormonal therapy including tamoxifen, GnRHa, can be adjuvant treatment after operation but it needs further research. Conflict of interest There are no conflicts of interest of all the authors and any kinds of supports for research, including funding, equipment, and drugs.
References 1. Birch-Hirschfeld FV (1896) Lehrbuch der pathologischen anatomie, 5th edn. East Germany, FCWVogel, Leipzig 2. Lam PM, Keith WK, Yu MY (2004) Intravenous leiomyomatosis: two cases with different routes of tumor extension. J Vasc Surg 39:465–469 3. Knauer E (1903) Beitrag zur anatomie der uterusmyome. Beitr Geburtsh Gynaekol 1:695 4. Sitzenfry A (1911) Ueber venenmyome des uterus mit intravaskularemwachstum. Z Gerburtsh Gynaekol 68:1–25
13
5. Kokawa K, Yamoto M, Yata C et al (2002) Postmenopausal intravenous leiomyomatosis with high levels of estrodiol and estrogen receptor. Obstet Gynecol 100:1124–1126 (5Pt2) 6. Dal CP, Quade BJ, Neskey DM et al (2003) Intravenous leiomyomatosis is characterized by a der (14) t (12; 14) (q15; q24). Genes Chromonsomes Cancer 36:205–206 7. Rispoli P, Santovito D, Tallia C et al (2010) A one-stage approach to the treatment of intravenous leiomyomatosis extending to the right heart. J Vasc Surg 52:212–215 8. Luciani N, Anselmi A, Glieca F et al (2009) Diagnostic and surgical issues in emergency presentation of a pelvic leiomyoma in the right heart. Ann Thorac Surg 87:1589–1592 9. Davidson T, Goitein O, Avigdor A et al (2009) 18F-FDG–PET/ CT for the diagnosis of tumor thrombosis. Isr Med Assoc J 12:69–73 10. Worley MJ Jr, Aelion A, Caputo TA et al (2009) Intravenous leiomyomatos is with intracardiac extension: a single-institution experience. Am J Obstet Gynecol 201:574.e1–574.e5 11. Ozer N, Engin H, Akgul E et al (2005) An unusual case of recurrent mass in the right atrium: intravenous leiomyomatosis. Echocardiography 22:514–516 12. Esmaeilzadeh M, Tavakolli A, Safaei A (2007) Recurrent intracardiac leiomyomatosis. Can J Cardiol 23:1085–1086 13. Clay TD, Dimitriou J, McNally OM et al (2013) Intravenous leiomyomatosis with intracardiac extension—a review of diagnosis and management with an illustrative case. Surg Oncol 22:44–52
Arch Gynecol Obstet 14. Vural Ç, Özen Ö, Demirhan B (2011) Intravenous lipoleiomyomatosis of uterus with cardiac extension: a case report. Pathol Res Pract 207:131–134 15. Marom D, Pitlik S, Sagie A et al (1998) Intravenous leiomyomatosis with cardiac involvement in a pregnant woman. Am J Obstet Gynecol 178:620–621 16. Tresukosol D, Kudelka AP, Malpica A et al (1995) Leuprolide acetate and intravascular leiomyomatosis. Obstet Gynecol 86(4 Pt 2):688–692
17. Mitsuhashi A, Nagai Y, Sugita M et al (1999) GnRH agonist for intravenous leiomyomatosis with cardiac extension. A case report. Reprod Med 44:883–886 18. Hameleers JA, Zeebregts CJ, Hamerlijnck RP et al (1999) Combined surgical and medical approach to intravenous leiomyomatosis with cardiac extension. Acta Chir Belg 99:92–94 19. Nam MS, Jeon MJ, Kim YT et al (2003) Pelvic leiomyomatosis with intracaval and intracardiac extension: a case report and review of the literature. Gynecol Oncol 89:175–180
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Case Report
Intravenous leiomyomatosis with intracardiac involvement Meng Xia · Junxiu Liu · Xianhong Xiang · Ming Xu · Mian He
Received: 15 March 2014 / Accepted: 28 April 2014 © Springer-Verlag Berlin Heidelberg 2014
Abstract Intravenous leiomyomatosis with intracardiac involvement is rare. This is a case report of a 52-year-old female with intravenous leiomyomatosis with intracardiac involvement. She was successfully treated with myomatectomy (left renal vein and inferior vena cava), hysterectomy, and bilateral salpingo-oophorectomy under the cardiopulmonary bypass. Keywords Intravenous leiomyomatosis · Intracardiac involvement
Introduction Intravenous leiomyomatosis is rare. Since the first description by Birch in 1896 [1], about 200 cases of intravenous leiomyomatosis have been reported in the literatures. Among these cases, about 10–30 % involve inferior vena cava, lung and even the cardiac cavity [2]. The first case of intravenous leiomyomatosis with intracardiac involvement was also described by Birch in 1906 [1]. So far, about 78 cases have been reported in the literatures.
M. Xia · J. Liu · M. He (*) Department of Gynecology and Obstetrics, The First Affiliated Hospital of Sun YAT-sen University, Guangzhou 510080, China e-mail: [email protected] X. Xiang Department of Interventional Radiology, The First Affiliated Hospital of Sun YAT-sen University, Guangzhou 510080, China M. Xu Department of Ultrasound, The First Affiliated Hospital of Sun YAT-sen University, Guangzhou 510080, China
Case report A 63-year-old female presented with a year of recurrent chest tightness, syncope and abdominal distention. A month prior to admitting to hospital, she received duplex ultrasound examination. It demonstrated the deep venous thrombosis of left lower extremity involving left renal vein and inferior vena cava. She denied any other symptoms, history of surgery, or vascular disease. She received hormone therapy for abnormal uterine bleeding in 2007 and has 2-year history of uterine myoma. On general physical exam, there was no abnormal founding. Laboratory values were in normal range including blood routine, liver and renal functions. A general PET-CT scan revealed multiple hypo dense lesions in the uterus body, which had irregular shape, unclear border. The biggest lesion was located in the left side of uterine body, and the range was 4.0 × 2.0 × 3.2 cm. Lesions involved the left adnexal and the broad ligament of the uterus. From the PET-CT images, it was found that left ovarian vein, left renal vein and inferior vena cava were augmented and lumen showed low dense images. It was inferred that the uterine leiomyomatosis extended to inferior vena cava through the left ovarian vein and left renal vein, and reached right atrium. Plain, contrast-enhanced and three-dimensional reconstruction spiral CT scans showed the multiple abnormal density lesions of uterus, from which we concluded uterine leiomyomatosis spread to inferior vena cava through the left ovarian vein and left renal vein, and reached right atrium (Fig. 1). Gynecological ultrasound showed multiple small uterine fibroids. Oval fluid dark space was found in the left adnexal, which was 35 × 26 mm. The patient was diagnosed as intravenous leiomyomatosis with intracardiac involvement. After multidisciplinary
13
Arch Gynecol Obstet
Fig. 1 Imaging examination before operation. a The PET-CT scan: the multiple abnormal density lesions of uterus, and the lesions extended to inferior vena cava through the left ovarian vein and left renal vein. The lesions had not increased metabolic abnormalities. b Contrast-enhanced CT scan: the left ovarian vein, left renal vein, inferior vena cava to atrium had filling defect
discussion, it was decided that operation was the best treatment. The patient underwent myomatectomy (left renal vein and inferior vena cava), hysterectomy, and bilateral salpingo-oophorectomy under the cardiopulmonary bypass. The tumor originated from uterine was cord-like, with medium texture and pale color. It floated in the vessels and spread from the uterine and ovarian veins to right atrium (Fig. 2). The tumor was resected totally with no visible residual. Successful operation was performed. The patient recovered well after operation and received regular followup. No episode of tumor recurrence was observed during the follow-up (Fig. 3).
Discussion The cause of intravenous leiomyomatosis is still unknown. There are two kinds of etiological theories: leiomyomatosis growing from vessel wall and leiomyomatosis growing into cardiac cavity along main vessels [3, 4]. In our case, the tumor spread from uterine vessel to cardiac cavity and was not adherently tight with the vessel wall. It seemed that our case supported the theory of leiomyomatosis growing into cardiac cavity along main vessels. Kokawa et al. [5] reported a postmenopausal patient with high levels of estradiol and estrogen receptor diagnosed intravenous leiomyomatosis. It indicated that high levels of estradiol and estrogen receptor were related to the growth and spreading of intravenous leiomyomatosis. In
13
our case, the patient received hormonal therapy for abnormal uterine bleeding with the test of estradiol 1,129 pg/ml. Dal et al. [6] found that there was chromosome abnormality of translocation between 12q15 and 14q24, although there was no similar abnormality in patients with uterine myoma. It indicated that the chromosome abnormality was also related to intravenous leiomyomatosis. Early diagnosis is difficult, because patients may be asymptomatic despite extensive intravenous extension or there are some mild, non-specific symptoms including dyspnea on exertion, shortness of breath, orthopnea, pleuritic chest pain, and syncopal episodes [7]. Both exact examinations and awareness of the disease are needed. Abdominal ultrasonography and a CT scan are usually used. Despite this, there are still a few patients misdiagnosed as tumor thrombosis and accepted anticoagulation therapy in earlier time [7, 8]. PET-CT is helpful for the diagnosis. 18F-FDGPET-CT scans can be used to differentiate between benign and malignant diseases such as tumor thrombosis, as the malignant disease has increased uptake of 18-FDG, whereas the benign exhibits normal biodistribution of the agent [9]. Successful therapy relies on surgical resection. In previous displays, complete surgical excision is often precluded by involvement of the tumor in the deep tributaries of the hypogastric vessels [10]. However, isolation of residual tumor appears equally important to prevent recurrences that can necessitate further surgery or lead to death. Recurrent disease is common and can occur up to 15 years after surgery [11]. Redevelopment can also occur quite rapidly.
Arch Gynecol Obstet Fig. 2 Resected tissues. a Uterus, bilateral adnexa and tumor embolus in left ovarian rein, b tumor embolus in inferior vena cava; pathological findings (×200). c Hematoxylin and eosin (HE) staining, d–h immunohistochemical (IHC) staining, vimentin (+), Actin (+), S-100 (+), endothelial cells CD31 (+), HMB45 (−), respectively
As these lesions are at great risk to recur, close long-term follow-up is essential [12]. In order to reduce the risk of future recurrence, most patients underwent hysterectomy, bilateral salpingo-oophorectomy. Another reason for undergoing the radical operation is the elder age. Age of onset for these patients concentrated in the 40–63 years of age and most of them have no fertility requirements [7, 13, 14]. However, patients may present with intravenous leiomyomatosis with intracardiac involvement from 40 years old or even younger. There was one case reported during pregnancy. Marom et al. [15]
reported a case in which the diagnosis was made at 8 weeks of gestation, followed by delivery by Cesarean section at 36 weeks. A two-stage operation was performed after delivery. But until now, there are no studies focusing on the issue of uterine preservation for the purpose of future fertility. Hormonal therapy is also used in the past decade. The use of postoperative antiestrogens, such as tamoxifen, GnRHa, has been considered, but their efficacy has yet to be established [16–19]. Long-term efficacy comparing choices in postoperative treatment is lacking but presents an area of interest for future investigations.
13
Arch Gynecol Obstet
Fig. 3 The CT scan before and after operation. a, b The CT scan before operation and c, d the CT scan on the 64th day after operation showed the left ovarian vein disappeared and the recanalization of left renal vein and Inferior vena cava
In conclusion, intravenous leiomyomatosis with intracardiac involvement is a rare disease that there are no specific presentations. Operation is the best treatment and total resection can prevent recurrence effectively. Hormonal therapy including tamoxifen, GnRHa, can be adjuvant treatment after operation but it needs further research. Conflict of interest There are no conflicts of interest of all the authors and any kinds of supports for research, including funding, equipment, and drugs.
References 1. Birch-Hirschfeld FV (1896) Lehrbuch der pathologischen anatomie, 5th edn. East Germany, FCWVogel, Leipzig 2. Lam PM, Keith WK, Yu MY (2004) Intravenous leiomyomatosis: two cases with different routes of tumor extension. J Vasc Surg 39:465–469 3. Knauer E (1903) Beitrag zur anatomie der uterusmyome. Beitr Geburtsh Gynaekol 1:695 4. Sitzenfry A (1911) Ueber venenmyome des uterus mit intravaskularemwachstum. Z Gerburtsh Gynaekol 68:1–25
13
5. Kokawa K, Yamoto M, Yata C et al (2002) Postmenopausal intravenous leiomyomatosis with high levels of estrodiol and estrogen receptor. Obstet Gynecol 100:1124–1126 (5Pt2) 6. Dal CP, Quade BJ, Neskey DM et al (2003) Intravenous leiomyomatosis is characterized by a der (14) t (12; 14) (q15; q24). Genes Chromonsomes Cancer 36:205–206 7. Rispoli P, Santovito D, Tallia C et al (2010) A one-stage approach to the treatment of intravenous leiomyomatosis extending to the right heart. J Vasc Surg 52:212–215 8. Luciani N, Anselmi A, Glieca F et al (2009) Diagnostic and surgical issues in emergency presentation of a pelvic leiomyoma in the right heart. Ann Thorac Surg 87:1589–1592 9. Davidson T, Goitein O, Avigdor A et al (2009) 18F-FDG–PET/ CT for the diagnosis of tumor thrombosis. Isr Med Assoc J 12:69–73 10. Worley MJ Jr, Aelion A, Caputo TA et al (2009) Intravenous leiomyomatos is with intracardiac extension: a single-institution experience. Am J Obstet Gynecol 201:574.e1–574.e5 11. Ozer N, Engin H, Akgul E et al (2005) An unusual case of recurrent mass in the right atrium: intravenous leiomyomatosis. Echocardiography 22:514–516 12. Esmaeilzadeh M, Tavakolli A, Safaei A (2007) Recurrent intracardiac leiomyomatosis. Can J Cardiol 23:1085–1086 13. Clay TD, Dimitriou J, McNally OM et al (2013) Intravenous leiomyomatosis with intracardiac extension—a review of diagnosis and management with an illustrative case. Surg Oncol 22:44–52
Arch Gynecol Obstet 14. Vural Ç, Özen Ö, Demirhan B (2011) Intravenous lipoleiomyomatosis of uterus with cardiac extension: a case report. Pathol Res Pract 207:131–134 15. Marom D, Pitlik S, Sagie A et al (1998) Intravenous leiomyomatosis with cardiac involvement in a pregnant woman. Am J Obstet Gynecol 178:620–621 16. Tresukosol D, Kudelka AP, Malpica A et al (1995) Leuprolide acetate and intravascular leiomyomatosis. Obstet Gynecol 86(4 Pt 2):688–692
17. Mitsuhashi A, Nagai Y, Sugita M et al (1999) GnRH agonist for intravenous leiomyomatosis with cardiac extension. A case report. Reprod Med 44:883–886 18. Hameleers JA, Zeebregts CJ, Hamerlijnck RP et al (1999) Combined surgical and medical approach to intravenous leiomyomatosis with cardiac extension. Acta Chir Belg 99:92–94 19. Nam MS, Jeon MJ, Kim YT et al (2003) Pelvic leiomyomatosis with intracaval and intracardiac extension: a case report and review of the literature. Gynecol Oncol 89:175–180
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