|Year : 2020 | Volume
| Issue : 3 | Page : 294-296
Deepak George John, Krishna Muralitharan, K S Saravana Krushna Raja, N Sritharan
Department of Vascular Surgery, Institute of Vascular Surgery, Madras Medical College and Rajiv Gandhi Government General Hospital, Chennai, Tamil Nadu, India
|Date of Submission||06-Dec-2019|
|Date of Acceptance||10-Dec-2019|
|Date of Web Publication||12-Sep-2020|
Deepak George John
Department of Vascular Surgery, Institute of Vascular Surgery, Madras Medical College and Rajiv Gandhi Government General Hospital, Chennai, Tamil Nadu
Source of Support: None, Conflict of Interest: None
Intravenous leiomyomatosis is a rare clinical entity which arises from the uterus and extends into the venous system reaching up to the inferior vena cava (IVC), right atrium (RA), and pulmonary vasculature. Very few cases have been reported with intracardiac extension. Complete resection of the tumor is essential for favorable outcomes. However, this requires a multidisciplinary approach with appropriate imaging and planning. We report a case of uterine leiomyomatosis with extension to the IVC, RA, right ventricle, and superior vena cava.
Keywords: Cardiac extension, inferior vena cava, intravenous, leiomyomatosis, superior venacava
|How to cite this article:|
John DG, Muralitharan K, Raja K S, Sritharan N. Intravenous leiomyomatosis. Indian J Vasc Endovasc Surg 2020;7:294-6
| Introduction|| |
Intravenous leiomyomatosis is a rare clinical entity, first described by Birch–Hirschfield in 1896. Extension of the tumor masses into the inferior vena cava (IVC) have been described, with a little more than 200 cases having been reported regarding this clinical entity. Even rarer is an extension to the right ventricle or pulmonary metastases. Up to 78 cases of intravenous leiomyomatosis with extension to the heart have been described, with about eight cases showing extension to the right ventricle.
We encountered such a case in our clinical practice, and took her up for debulking with tumor thrombus removal, in collaboration with the cardiothoracic and surgical oncology teams. The outcome was favorable and the management of this case adds significant knowledge to this rare and potentially fatal entity.
| Case Report|| |
A 36-year-old female presented to the surgical oncology department with an abdominal swelling for 1-year, which was gradually increasing in size. She had no vomiting, abdominal distension, loss of appetite or weight, hematochezia, or bleeding per rectum. She did not have any features suggestive of bowel obstruction. Her menstrual cycles were irregular.
On examination, there was mass of size 15 cm × 20 cm with well-defined borders mainly involving the lower abdomen extending above the umbilicus. It was not possible to get below the swelling. It was nontender and nonpulsatile. All peripheral pulses were normal, and there was no lower limb swelling. The rest of the systemic examination was within the normal limits.
Imaging revealed solid mass (20 cm × 18 cm × 8 cm) arising from the pelvis causing compression on the bladder, resulting in bilateral hydroureteronephrosis. There was extension of the same mass into the right iliac vein and extending into the IVC and right atrium (RA) [Figure 1]. The patient was taken up for surgery by the surgical oncology team with the vascular surgical team and cardio thoracic surgical team assisting. Sterno-laparotomy was done. The mass was completely resected first [Figure 2], which was followed by cardiopulmonary bypass (CPB) with cannulation of the ascending aorta, superior vena cava (SVC), and right atrial appendage. Subsequently, hypothermia (26°C), circulatory arrest and right atriotomy were done by the thoracic team. The fleshy mass was seen extending into the RA, SVC and right ventricle [Figure 3]. Subsequently, cavotomy of the IVC at infra renal segment was performed. The mass was then partly extracted from the atrium and the remainder was extracted from the IVC [Figure 4]. The atriotomy and cavotomy were closed, and circulation was restored. Following this, patient started to develop bleeding from the pelvic veins. Attempts to control the bleed were unsuccessful, and hence, the pelvis was packed with gauze pads and the abdomen was closed. Once she was stabilized hemodynamically, she was taken for relaparotomy after 48 h. Pads were removed bleeding was controlled. Wash was given, and abdominal closure was done.
|Figure 1:Imaging showing the mass with inferior vena cava and cardiac extension and cut sections delineating the intraluminal thrombus|
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|Figure 3: Intraoperative picture showing the intraluminal mass with extension into the right atrium and ventricle|
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|Figure 4: Specimen following complete removal of the tumor from within Iliac veins, inferior vena cava, right atrium and ventricle|
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Patient was monitored in the ICU and was extubated on the 5th day, and she was discharged on the 14th day. The histopathological report was uterine leiomyomatosis with intravenous extension.
| Discussion|| |
Leiomyomas are the most common benign tumors arising in the uterus. They have a tendency to spread to extrauterine site, most commonly pelvic veins or peritoneal cavity. Rarely, the tumors may spread to the IVC and extend to the RA. Even rarer is an extension to the right ventricle or pulmonary metastases. Up to 78 cases of intravenous leiomyomatosis with extension to the heart have been described, with about eight cases showing extension to the right ventricle. Pulmonary metastases, also known as benign metastatic leiomyoma, are histologically benign, but clinically aggressive lesions within the pulmonary parenchyma.
The prevailing theories regarding the pathogenesis of intravenous leiomyomatosis are either (1) Origin of the leiomyomatous tissue from within the veins walls and intraluminal spread, or (2) Extension of leiomymatous tissue from uterine leiomyomas or remnants in posthysterectomy patients.,
The tumors were described as spiral or nodular within the pelvic veins. Intraluminal extension was hypothesized to be by stretching the vascular channels while covered by endothelium rather than by infiltration. The spread is usually through the internal iliac vein, common iliac vein to the IVC and right heart with or without extension to the pulmonary arteries or lung parenchyma. There have been cases with extension into the gonadal vein.
Literature review showed the presence of about 200 case reports of intravenous leiomyomatosis with varying extent of tumor thrombus., Only eight cases reported to have extension into the right ventricle with three showing extension into the pulmonary artery.
Patients with intravenous leiomyomatosis are often asymptomatic but may present with abdominal distension, ascites, hematuria, lower-extremity deep venous thrombosis or chest pain, dyspnea, or even right heart failure The diagnosis is often missed as the patients may present at an advanced stage; and dyspnea; right heart failure may not be immediately be correlated with this diagnosis.
Patients with benign metastasizing leiomyoma may either remain asymptomatic and diagnosed incidentally, or cause, cough, chest pain, dyspnea, or hemoptysis.
The evaluation of the condition is usually either with a contrast-enhanced computed tomography (CT) or magnetic resonance imaging (MRI) scan. The high-density resolution of CT imaging as well as the soft-tissue delineation on MRI renders these modalities as the ideal for imaging of the intraluminal tumors. The signal intensity on MRI imaging depends on the number of smooth muscle cells or the presence of hyalinized fibrous tissue within the vessels.
The majority of such cases can be taken up as a single-stage or 2-stage procedure. A single-stage procedure involves removal of the primary source with total abdominal hysterectomy, bilateral salpingo-oophorectomy, with cavotomy with the removal of intraluminal thrombus for cases with IVC extension alone. For cases with cardiac extension, CPB with cannulation of the aorta, SVC, and right atrial appendage, followed by the right atriotomy and removal of thrombus were done at the same setting.
For a 2 stage operation, the abdominal and the cardiac tumor thrombus were dealt with in separate settings.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]