- Email: [email protected]
Intravascular Leiomyomatosis Extending to Right Atrium: A Rare Caused Syncope
Journal Pre-proof Intravascular leiomyomatosis extending to right atrium: a rare caused syncope Yingjiang Xu, Xiujuan Gao, Chao Yang, Jianyong Liu, Bi Jin, Dan Shang PII:
S0890-5096(19)30986-0
DOI:
https://doi.org/10.1016/j.avsg.2019.11.024
Reference:
AVSG 4787
To appear in:
Annals of Vascular Surgery
Received Date: 20 August 2019 Revised Date:
18 October 2019
Accepted Date: 10 November 2019
Please cite this article as: Xu Y, Gao X, Yang C, Liu J, Jin B, Shang D, Intravascular leiomyomatosis extending to right atrium: a rare caused syncope, Annals of Vascular Surgery (2019), doi: https:// doi.org/10.1016/j.avsg.2019.11.024. This is a PDF file of an article that has undergone enhancements after acceptance, such as the addition of a cover page and metadata, and formatting for readability, but it is not yet the definitive version of record. This version will undergo additional copyediting, typesetting and review before it is published in its final form, but we are providing this version to give early visibility of the article. Please note that, during the production process, errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. © 2019 Published by Elsevier Inc.
1
Intravascular leiomyomatosis extending to right atrium: a rare
2
caused syncope
3
Yingjiang Xu1,2, Xiujuan Gao3, Chao Yang4, Jianyong Liu4, Bi Jin4, Dan Shang4,*
4
1
5
Binzhou, Shandong Province, People's Republic of China
6
2
7
Department of Biochemistry and Molecular Biology, Fudan University Shanghai Medical
8
College, Shanghai, People's Republic of China
9
3
Department of Interventional Vascular Surgery, Binzhou Medical University Hospital,
Key Laboratory of Metabolism and Molecular Medicine, The Ministry of Education,
Department of Cerebrovascular Neurosurgery, Binzhou Medical University Hospital,
10
Binzhou, Shandong Province, People's Republic of China
11
4
12
University of Science and Technology, Wuhan, Hubei Province, People's Republic of
13
China
14
Yingjiang Xu and Xiujuan Gao contributed equally to this work.
15
Correspondent author ： Dan Shang, Email: [email protected], Department of
16
Vascular Surgery, Union Hospital, Tongji Medical College, Huazhong University of
17
Science and Technology, 1277 Jiefang Avenue, Wuhan, Hubei Province, 430022, China.
18
Tele: +8615902708478.
19
Abstract
Department of Vascular Surgery, Union Hospital, Tongji Medical College, Huazhong
20
Intravascular leiomyomatosis (IVL) is a variant of leiomyoma characterized by
21
intravascular proliferation of a histologically benign smooth muscle tumor extending
22
beyond the uterus into distant great vessels or the heart. It is a rare disease and results in
23
death. Here, we reported the case of 48-year-old, otherwise well woman, who presented
24
to the emergency department with syncope. Pulmonary CT demonstrated a large
25
low-density shadow originating from the inferior vena cava (IVC) extending into the right
26
atrium (RA). MRV showed that a neoplasm was "snakelike" which completely occluded
27
the right internal iliac vein (RIIV), the common iliac vein (CIV), and IVC.A multidisciplinary
28
team of specialists consisting of vascular surgeons, cardiac surgeons, gynecologists,
29
anesthesiologists, and radiologists reviewed the history, clinical examination findings, and
30
diagnostic imaging of the patient. A decision was made to proceed with one-stage surgery
31
(resection of thoracoabdominal tumor extension at one operative setting).After surgery,
32
the patient's vital signs were restored and her symptoms were disappeared. She was
33
discharged on hospital day 21 without complications. One-stage surgical approach to
34
completely remove an IVL with RA involvement is an optimal choice if the patient's
35
physical condition permits.
36
Keywords: Syncope; Intravenous leiomyomatosis; Inferior vena cava
37
Intravenous leiomyomatosis (IVL) is a rare condition, which is a benign smooth
38
muscle tumor most often arising from the uterus and spreads through pelvic veins
39
extending as far as the heart, via the inferior vena cava (IVC).1 IVL has a benign histology
40
with malignant behavior and can result in syncope if left untreated as it blocks the venous
41
return. In the case presented herein, we successfully treated syncope caused by IVL
42
utilizing a one-stage surgery combining sternotomy and laparotomy.
43
Case presentation
44
A 48-year-old, otherwise well woman, presented with exertional recurrent syncopal
45
episodes for approximately 5 months. The above symptoms worsened over a 1-hour
46
period accompanied by loss of consciousness. A diastolic rumble was heard on the left
47
third and fourth intercostal space when the patient was sitting down and a fist-sized mass
48
in the right adnexal area was palpated during a gynecological examination. The patient's
49
vital signs were stable. A preliminary pulmonary CT was performed in a local medical
50
center 1-weeks ago, which demonstrated a large low-density shadow originating from the
51
IVC extending into the right atrium(RA)(Fig.1A–B).
52
She was investigated for an underlying cause in our medical center. Workup included
53
screening bloods, B-mode ultrasound imaging of the abdominal vena cava and pelvis, and
54
magnetic resonance venography (MRV). Routine bloods revealed mild anaemia with
55
haemoglobin of 97 g/L and a mildly elevated D-dimer of 0.70 mg/L (reference range in our
56
laboratory 0–0.50). Other laboratory test revealed normal. B-mode ultrasound imaging
57
can detect cord-like structures with mild heterogeneous echogenicity within the IVC and
58
RA. MRV showed that a neoplasm was "snakelike" which completely occluded the right
59
internal iliac vein (RIIV), the common iliac vein (CIV), and IVC (Fig. 1C–D).
60
A multidisciplinary team of specialists consisting of vascular surgeons, cardiac
61
surgeons, gynecologists, anesthesiologists, and radiologists reviewed the history, clinical
62
examination findings, and diagnostic imaging of the patient. A decision was made to
63
proceed with one-stage surgery (resection of thoracoabdominal tumor extension at one
64
operative setting).Gynecologists as the first-stage procedures were performed total
65
hysterectomy and bilateral salpingohysterectomy under general anesthetic. A mass of
66
5cm*6cm*6cm was resected from the pelvic cavity (Fig. 2A). The histological images
67
(hematoxylin and eosin) revealed fusiform smooth muscle cells arranged in a bundle and
68
vascular hyaline degeneration in the tumor, which consistent with intrauterine
69
leiomyomatosis (Fig. 2B). Next, cardiopulmonary bypass (CPB) was established by a
70
cardiac surgeon via cannulation of the superior vena cava, the IVC, and the ascending
71
aorta. After the proximal and distal of the tumor were controlled, the vascular surgeon
72
incised the IVC longitudinally at the iliac bifurcation to separate the adherent intravascular
73
tumor from the vascular wall sufficiently (Fig. 2C).Since the incision was small and the IVC
74
wall defect is less than 1/4 of the vessel circumference, the inferior vena cava was
75
repaired with simple suture.The atria was also repaired by simple suture.Following a right
76
atriotomy, an unusual cream-coloured, sausage-like material was manually extracted out
77
of the IVC through the RA (Fig. 2D).2The patient tolerated the course well, and the
78
procedure was smooth without complications. Gross specimen was firm, regularly shaped
79
measuring up to 20cm in length (Fig. 2E). The histology of the mass showed a smooth
80
muscle tumor which was positive for desmin, smooth muscle actin, and progesterone and
81
estrogen receptors consistenting with an IVL (Fig. 2F).Twelve months following surgery,
82
she remains asymptomatic with no further syncope(Fig. 1E–F).
83
Discussion
84
IVL is a rare mesodermal cell tumor with two hypotheses, which are derived from
85
smooth muscle in uterine vascular wall and invasion of venous endarterium by uterine
86
fibroids.3 Previous reports indicate that IVL is associated with uterine leiomyoma.
87
However, our center has reported a case of subclavian leiomyomatosis.4 The disease
88
often conceals onset, from the primary site through the uterus or pelvic vein, renal vein,
89
vena cava, and finally into the RA, and even into the right ventricle, pulmonary artery
90
through the tricuspid valve, resulting in sudden death. IVL with intracardiac extension was
91
first reported in 1907,5 and since then, many cases have been documented in the
92
literature, however, all of which no more than 100 cases involving the right heart system.
93
All the patients were women, with an average age of 47 years. Most of them were
94
menopausal or postpartum women.6
95
The clinical manifestations of IVL are closely related to the extent of involvement.
96
When the lesion was confined to the uterus, it was only manifested as abdominal pain,
97
bleeding, increased leucorrhea, abdominal mass and compression symptoms; involving
98
the inferior vena cava, ascites, hepatosplenomegaly, lower limb edema may occur. A few
99
involve the right atrium, which can cause palpitation, dyspnea and syncope. IVL diagnosis
100
requires a thorough understanding of the clinician. B-ultrasound, CT and MRV allow
101
prompt diagnosis of the condition, however, it suggests that MRI be the optimal choice.
102
Patients diagnosed with IVL should be actively treated with surgery due to result in
103
causing fatal cardiovascular complications. Complete surgical resection is the treatment
104
of choice for IVL.7 However, IVL resection includes thoracic and pelvic-abdominal surgery,
105
which can be performed a one-stage in the cardiopulmonary bypass, or two-stage
106
operation at intervals of 4 to 6 weeks. Wang et al. found that compared with the one-stage
107
operation, the two-stage operation had a longer overall operation time, a larger amount of
108
total bleeding and a longer total hospital stay.8 Therefore, the selection of surgical options
109
should be based on the patient's general condition. In general, patients who are in good
110
condition and can tolerate extensive surgery should be resected in one-stage. On the
111
contrary, when liver function is obviously impaired and a large amount of ascites occurs.
112
Intracardiac mass should be resected preferentially. In the case of our patient, the patient
113
has no underlying disease. Meanwhile, the intracardiac part of IVL was freely movable
114
and the maximum diameter was larger than that of the IVC. Thus, removal of both
115
intracardiac and intravascular parts of the IVL via RA was planned in one-stage.
116
Conclusion
117
In this case report, we describe a rare syncope caused by intravascular
118
leiomyomatosis. One-stage surgical approach to completely remove an IVL with RA
119
involvement is an optimal choice if the patient's physical condition permits.
120
Acknowledgements
121 122
The authors thank the patient for permitting to use patient's data. Funding
123
Not available.
124
Conflict of interest
125 126 127
The authors have no funding and conflicts of interest to disclose. Ethical approval Ethical approval for this study was approved by the medical committee of Union
128
Hospital, Tongji Medical College, Huazhong University of Science and Technology.
129
References
130
1.Barksdale J, Abolhoda A, Saremi F. Intravenous leiomyomatosis presenting as acute 1.
131
Budd-Chiari syndrome. J Vasc Surg 2011; 54: 860-3.
132
2.Yang C, Fang H, Yang Y, et al. Diagnosis and surgical management of inferior vena 2.
133
cava leiomyomatosis. J Vasc Surg-Venous L 2018; 6: 636-45.
134
3.Sitzenfry A. Über Venenmyome des Uterus mit intravaskularem Wachstum. Z 3.
135
Geburtshilfe Gynaekol 1911;68:1-25.
136
4.Fang H, You Y, Cai F. Intravenous leiomyomatosis of the subclavian vein. J Vasc 4.
137
Surg-Venous L 2017; 5: 254-6.
138
5.Durck 5. H. Über ein kontinuierlich durch die untere Hohlvene in das Herz vorwachsendes:
139
Fibromyom des Uterus. Münch Med Wochenschr1907;54:1154.
140
6.Yu 6. X, Zhang G, Lang J, et al. Factors associated with recurrence after surgical resection
141
in women with intravenous leiomyomatosis. Obstet Gynecol 2016;128:1018-24.
142
7.Castelli P, Caronno R, Piffaretti G, et al. Intravenous uterine leiomyomatosis with right 7.
143
heart extension: successful two-stage surgical removal. Ann Vasc Surg 2006;20:405–7.
144
8.Wang J, Yang J, Huang H, et al. Management of intravenousleiomyomatosis with 8.
145
intracaval and intracardiac extension. Obstet Gynecol, 2012, 120:1400-6.
146 147
Figure 1. (A, B) Computed tomography showed a large low-density shadow extending from inferior vena
148
cava to the right atrium. (C) MRV showed that a neoplasm was "snakelike" which completely occluded
149
the right internal iliac vein, the common iliac vein, and inferior vena cava. (D) The inferior vena cava was
150
occluded. (E,F) CT venography were followed up 12 months after surgery.IVC=inferior vena cava,
151
RA=right atrium,RV=right ventricle,LV=left ventricle,AT=thoracic artery.
152 153
Figure 2.(A) First, pelvic lumpectomy was performed by a gynecologist via a midline laparotomy.(B) 2
154
Pelvic neoplasms was pathologically confirmed with intrauterine leiomyomatosis (original magnification
155
×100). (C) IVC longitudinally was incised at the iliac bifurcation to separate the adherent intravascular
156
tumor from the vascular wall sufficiently.(D) Tumor tissue was manually extracted out of the IVC through
157
the right atriotomy. (E) Gross specimen was firm, regularly shaped measuring up to 20cm in length.(F)
158
The histology of the mass showed a smooth muscle tumor which was positive for desmin, smooth muscle
159
actin, and progesterone and estrogen receptors consistenting with an IVL (original magnification ×100).
S0890-5096(19)30986-0
DOI:
https://doi.org/10.1016/j.avsg.2019.11.024
Reference:
AVSG 4787
To appear in:
Annals of Vascular Surgery
Received Date: 20 August 2019 Revised Date:
18 October 2019
Accepted Date: 10 November 2019
Please cite this article as: Xu Y, Gao X, Yang C, Liu J, Jin B, Shang D, Intravascular leiomyomatosis extending to right atrium: a rare caused syncope, Annals of Vascular Surgery (2019), doi: https:// doi.org/10.1016/j.avsg.2019.11.024. This is a PDF file of an article that has undergone enhancements after acceptance, such as the addition of a cover page and metadata, and formatting for readability, but it is not yet the definitive version of record. This version will undergo additional copyediting, typesetting and review before it is published in its final form, but we are providing this version to give early visibility of the article. Please note that, during the production process, errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. © 2019 Published by Elsevier Inc.
1
Intravascular leiomyomatosis extending to right atrium: a rare
2
caused syncope
3
Yingjiang Xu1,2, Xiujuan Gao3, Chao Yang4, Jianyong Liu4, Bi Jin4, Dan Shang4,*
4
1
5
Binzhou, Shandong Province, People's Republic of China
6
2
7
Department of Biochemistry and Molecular Biology, Fudan University Shanghai Medical
8
College, Shanghai, People's Republic of China
9
3
Department of Interventional Vascular Surgery, Binzhou Medical University Hospital,
Key Laboratory of Metabolism and Molecular Medicine, The Ministry of Education,
Department of Cerebrovascular Neurosurgery, Binzhou Medical University Hospital,
10
Binzhou, Shandong Province, People's Republic of China
11
4
12
University of Science and Technology, Wuhan, Hubei Province, People's Republic of
13
China
14
Yingjiang Xu and Xiujuan Gao contributed equally to this work.
15
Correspondent author ： Dan Shang, Email: [email protected], Department of
16
Vascular Surgery, Union Hospital, Tongji Medical College, Huazhong University of
17
Science and Technology, 1277 Jiefang Avenue, Wuhan, Hubei Province, 430022, China.
18
Tele: +8615902708478.
19
Abstract
Department of Vascular Surgery, Union Hospital, Tongji Medical College, Huazhong
20
Intravascular leiomyomatosis (IVL) is a variant of leiomyoma characterized by
21
intravascular proliferation of a histologically benign smooth muscle tumor extending
22
beyond the uterus into distant great vessels or the heart. It is a rare disease and results in
23
death. Here, we reported the case of 48-year-old, otherwise well woman, who presented
24
to the emergency department with syncope. Pulmonary CT demonstrated a large
25
low-density shadow originating from the inferior vena cava (IVC) extending into the right
26
atrium (RA). MRV showed that a neoplasm was "snakelike" which completely occluded
27
the right internal iliac vein (RIIV), the common iliac vein (CIV), and IVC.A multidisciplinary
28
team of specialists consisting of vascular surgeons, cardiac surgeons, gynecologists,
29
anesthesiologists, and radiologists reviewed the history, clinical examination findings, and
30
diagnostic imaging of the patient. A decision was made to proceed with one-stage surgery
31
(resection of thoracoabdominal tumor extension at one operative setting).After surgery,
32
the patient's vital signs were restored and her symptoms were disappeared. She was
33
discharged on hospital day 21 without complications. One-stage surgical approach to
34
completely remove an IVL with RA involvement is an optimal choice if the patient's
35
physical condition permits.
36
Keywords: Syncope; Intravenous leiomyomatosis; Inferior vena cava
37
Intravenous leiomyomatosis (IVL) is a rare condition, which is a benign smooth
38
muscle tumor most often arising from the uterus and spreads through pelvic veins
39
extending as far as the heart, via the inferior vena cava (IVC).1 IVL has a benign histology
40
with malignant behavior and can result in syncope if left untreated as it blocks the venous
41
return. In the case presented herein, we successfully treated syncope caused by IVL
42
utilizing a one-stage surgery combining sternotomy and laparotomy.
43
Case presentation
44
A 48-year-old, otherwise well woman, presented with exertional recurrent syncopal
45
episodes for approximately 5 months. The above symptoms worsened over a 1-hour
46
period accompanied by loss of consciousness. A diastolic rumble was heard on the left
47
third and fourth intercostal space when the patient was sitting down and a fist-sized mass
48
in the right adnexal area was palpated during a gynecological examination. The patient's
49
vital signs were stable. A preliminary pulmonary CT was performed in a local medical
50
center 1-weeks ago, which demonstrated a large low-density shadow originating from the
51
IVC extending into the right atrium(RA)(Fig.1A–B).
52
She was investigated for an underlying cause in our medical center. Workup included
53
screening bloods, B-mode ultrasound imaging of the abdominal vena cava and pelvis, and
54
magnetic resonance venography (MRV). Routine bloods revealed mild anaemia with
55
haemoglobin of 97 g/L and a mildly elevated D-dimer of 0.70 mg/L (reference range in our
56
laboratory 0–0.50). Other laboratory test revealed normal. B-mode ultrasound imaging
57
can detect cord-like structures with mild heterogeneous echogenicity within the IVC and
58
RA. MRV showed that a neoplasm was "snakelike" which completely occluded the right
59
internal iliac vein (RIIV), the common iliac vein (CIV), and IVC (Fig. 1C–D).
60
A multidisciplinary team of specialists consisting of vascular surgeons, cardiac
61
surgeons, gynecologists, anesthesiologists, and radiologists reviewed the history, clinical
62
examination findings, and diagnostic imaging of the patient. A decision was made to
63
proceed with one-stage surgery (resection of thoracoabdominal tumor extension at one
64
operative setting).Gynecologists as the first-stage procedures were performed total
65
hysterectomy and bilateral salpingohysterectomy under general anesthetic. A mass of
66
5cm*6cm*6cm was resected from the pelvic cavity (Fig. 2A). The histological images
67
(hematoxylin and eosin) revealed fusiform smooth muscle cells arranged in a bundle and
68
vascular hyaline degeneration in the tumor, which consistent with intrauterine
69
leiomyomatosis (Fig. 2B). Next, cardiopulmonary bypass (CPB) was established by a
70
cardiac surgeon via cannulation of the superior vena cava, the IVC, and the ascending
71
aorta. After the proximal and distal of the tumor were controlled, the vascular surgeon
72
incised the IVC longitudinally at the iliac bifurcation to separate the adherent intravascular
73
tumor from the vascular wall sufficiently (Fig. 2C).Since the incision was small and the IVC
74
wall defect is less than 1/4 of the vessel circumference, the inferior vena cava was
75
repaired with simple suture.The atria was also repaired by simple suture.Following a right
76
atriotomy, an unusual cream-coloured, sausage-like material was manually extracted out
77
of the IVC through the RA (Fig. 2D).2The patient tolerated the course well, and the
78
procedure was smooth without complications. Gross specimen was firm, regularly shaped
79
measuring up to 20cm in length (Fig. 2E). The histology of the mass showed a smooth
80
muscle tumor which was positive for desmin, smooth muscle actin, and progesterone and
81
estrogen receptors consistenting with an IVL (Fig. 2F).Twelve months following surgery,
82
she remains asymptomatic with no further syncope(Fig. 1E–F).
83
Discussion
84
IVL is a rare mesodermal cell tumor with two hypotheses, which are derived from
85
smooth muscle in uterine vascular wall and invasion of venous endarterium by uterine
86
fibroids.3 Previous reports indicate that IVL is associated with uterine leiomyoma.
87
However, our center has reported a case of subclavian leiomyomatosis.4 The disease
88
often conceals onset, from the primary site through the uterus or pelvic vein, renal vein,
89
vena cava, and finally into the RA, and even into the right ventricle, pulmonary artery
90
through the tricuspid valve, resulting in sudden death. IVL with intracardiac extension was
91
first reported in 1907,5 and since then, many cases have been documented in the
92
literature, however, all of which no more than 100 cases involving the right heart system.
93
All the patients were women, with an average age of 47 years. Most of them were
94
menopausal or postpartum women.6
95
The clinical manifestations of IVL are closely related to the extent of involvement.
96
When the lesion was confined to the uterus, it was only manifested as abdominal pain,
97
bleeding, increased leucorrhea, abdominal mass and compression symptoms; involving
98
the inferior vena cava, ascites, hepatosplenomegaly, lower limb edema may occur. A few
99
involve the right atrium, which can cause palpitation, dyspnea and syncope. IVL diagnosis
100
requires a thorough understanding of the clinician. B-ultrasound, CT and MRV allow
101
prompt diagnosis of the condition, however, it suggests that MRI be the optimal choice.
102
Patients diagnosed with IVL should be actively treated with surgery due to result in
103
causing fatal cardiovascular complications. Complete surgical resection is the treatment
104
of choice for IVL.7 However, IVL resection includes thoracic and pelvic-abdominal surgery,
105
which can be performed a one-stage in the cardiopulmonary bypass, or two-stage
106
operation at intervals of 4 to 6 weeks. Wang et al. found that compared with the one-stage
107
operation, the two-stage operation had a longer overall operation time, a larger amount of
108
total bleeding and a longer total hospital stay.8 Therefore, the selection of surgical options
109
should be based on the patient's general condition. In general, patients who are in good
110
condition and can tolerate extensive surgery should be resected in one-stage. On the
111
contrary, when liver function is obviously impaired and a large amount of ascites occurs.
112
Intracardiac mass should be resected preferentially. In the case of our patient, the patient
113
has no underlying disease. Meanwhile, the intracardiac part of IVL was freely movable
114
and the maximum diameter was larger than that of the IVC. Thus, removal of both
115
intracardiac and intravascular parts of the IVL via RA was planned in one-stage.
116
Conclusion
117
In this case report, we describe a rare syncope caused by intravascular
118
leiomyomatosis. One-stage surgical approach to completely remove an IVL with RA
119
involvement is an optimal choice if the patient's physical condition permits.
120
Acknowledgements
121 122
The authors thank the patient for permitting to use patient's data. Funding
123
Not available.
124
Conflict of interest
125 126 127
The authors have no funding and conflicts of interest to disclose. Ethical approval Ethical approval for this study was approved by the medical committee of Union
128
Hospital, Tongji Medical College, Huazhong University of Science and Technology.
129
References
130
1.Barksdale J, Abolhoda A, Saremi F. Intravenous leiomyomatosis presenting as acute 1.
131
Budd-Chiari syndrome. J Vasc Surg 2011; 54: 860-3.
132
2.Yang C, Fang H, Yang Y, et al. Diagnosis and surgical management of inferior vena 2.
133
cava leiomyomatosis. J Vasc Surg-Venous L 2018; 6: 636-45.
134
3.Sitzenfry A. Über Venenmyome des Uterus mit intravaskularem Wachstum. Z 3.
135
Geburtshilfe Gynaekol 1911;68:1-25.
136
4.Fang H, You Y, Cai F. Intravenous leiomyomatosis of the subclavian vein. J Vasc 4.
137
Surg-Venous L 2017; 5: 254-6.
138
5.Durck 5. H. Über ein kontinuierlich durch die untere Hohlvene in das Herz vorwachsendes:
139
Fibromyom des Uterus. Münch Med Wochenschr1907;54:1154.
140
6.Yu 6. X, Zhang G, Lang J, et al. Factors associated with recurrence after surgical resection
141
in women with intravenous leiomyomatosis. Obstet Gynecol 2016;128:1018-24.
142
7.Castelli P, Caronno R, Piffaretti G, et al. Intravenous uterine leiomyomatosis with right 7.
143
heart extension: successful two-stage surgical removal. Ann Vasc Surg 2006;20:405–7.
144
8.Wang J, Yang J, Huang H, et al. Management of intravenousleiomyomatosis with 8.
145
intracaval and intracardiac extension. Obstet Gynecol, 2012, 120:1400-6.
146 147
Figure 1. (A, B) Computed tomography showed a large low-density shadow extending from inferior vena
148
cava to the right atrium. (C) MRV showed that a neoplasm was "snakelike" which completely occluded
149
the right internal iliac vein, the common iliac vein, and inferior vena cava. (D) The inferior vena cava was
150
occluded. (E,F) CT venography were followed up 12 months after surgery.IVC=inferior vena cava,
151
RA=right atrium,RV=right ventricle,LV=left ventricle,AT=thoracic artery.
152 153
Figure 2.(A) First, pelvic lumpectomy was performed by a gynecologist via a midline laparotomy.(B) 2
154
Pelvic neoplasms was pathologically confirmed with intrauterine leiomyomatosis (original magnification
155
×100). (C) IVC longitudinally was incised at the iliac bifurcation to separate the adherent intravascular
156
tumor from the vascular wall sufficiently.(D) Tumor tissue was manually extracted out of the IVC through
157
the right atriotomy. (E) Gross specimen was firm, regularly shaped measuring up to 20cm in length.(F)
158
The histology of the mass showed a smooth muscle tumor which was positive for desmin, smooth muscle
159
actin, and progesterone and estrogen receptors consistenting with an IVL (original magnification ×100).