Intravenous leiomyomatosis: the first reported case of intraoperative intracaval embolisation of tumour to the right atrium

Abstract

Intravenous leiomyomatosis is extremely rare. This case describes a 42-year-old woman who presented with abdominal distension, cyclical bloating and urinary retention. Preoperative imaging showed a multilobulated uterine mass. Following multidisciplinary team discussion, a complete staging surgery consisting of midline laparotomy, total hysterectomy and bilateral salpingo-oophrectomy was performed. Intraoperatively, a large multilobulated uterine mass was noted with engorgement of the infundibulopelvic ligaments due to intravascular extension of tumour. On removal of the uterus, the patient desaturated and became hypotensive. Intraoperative transoesophageal echocardiography revealed mass extending from the inferior vena cava (IVC) into the right atrium (RA). The cardiothoracic surgical team retrieved a worm-like mass extending from the IVC into the RA. Histopathological examination diagnosed a large uterine leiomyoma with intravenous leiomyomatosis. The mass from the RA was a bland spindle cell tumour which matched the uterine mass histopathologically. Intravenous leiomyomatosis is a rare variant of uterine leiomyoma. Although intracardiac extension has been described, this is the first case of intraoperative embolisation of pelvic tumour to the RA at hysterectomy.

Background

Uterine leiomyoma, a monoclonal expansion of smooth muscle cells of the myometrium,¹ is a common disorder of the uterus. Reported incidence ranges from 25% to 50%.^{2 3} Less commonly, leiomyomata have been known to extend beyond the uterus. They can be found within the broad ligament, ovary, peritoneum⁴ and extra-abdominal sites such as the lungs.⁵ Intravenous extension of the leiomyomatous process (intravenous leiomyomatosis) is exceedingly rare, with less than 150 cases reported in the literature.⁵ It is a benign histological variant of typical leiomyomata,^{6 7} with quasi-malignant behaviour resulting in intraluminal extension of smooth muscle cell tumour into the adjacent lymphatic or venous system.⁸ The condition is associated with elevated serum oestrogen levels, which is thought to be a pathogenic factor.⁹ Intracardiac extension of leiomyomatosis has been reported previously.^{10 11} In these reports, diagnosis is made through preoperative imaging demonstrating intravascular, worm-like projections from uterine leiomyomata.^{10 11} Case reports describe diagnosis of intravenous leiomyomatosis, with intracardiac extension resulting in symptoms of cardiac failure,¹² months after hysterectomy for uterine leiomyoma.¹²

Management of this rare condition has been reported on a case-by-case basis. Previous authors describe one-stage¹³ versus two-stage procedures to resect intracardiac tumour with intracaval resection.^{11 14} There is a significant risk of recurrence of intravenous leiomyomatosis,¹⁵ reportedly as high as 31%. The risk appears to be directly related to preoperative tumour volume and large pelvic vein involvement.¹⁶ To date, no cases of embolism of leiomyomatous tumour from the pelvic vasculature to the right atrium (RA) at hysterectomy have been described.

Case presentation

A 42-year-old woman was referred to the gynaecology outpatient department by her general practitioner with a 2-year history of abdominal distension. She described severe midcycle abdominal bloating, pain and urinary retention, with resolution of symptoms at onset of menstruation. She had a regular 28-day cycle, normal smear history and was parous with three children born by spontaneous vaginal delivery. She used the mirena coil for contraception for the past 9 years. A number of years prior to the index presentation she was diagnosed with a supraventricular tachycardia which was successfully treated with ablation, and mitral valve prolapse that did not require any ongoing cardiac surveillance.

Her symptoms of pelvic pain and recurrent urinary retention had become increasingly frequent and severe. Initially, she was treated for recurrent urinary tract infections, but the development of a palpable abdominal mass prompted a referral for urgent gynaecological assessment.

Investigations

Ultrasonography showed a 20 cm large confluent mass arising from the uterus with internal flow, in keeping with uterine leiomyomata. The patient was given ulipristal acetate for symptomatic relief. The patient was referred for outpatient MRI of the pelvis which showed a large lobulated abdominal mass measuring 25×15×30 cm, appearing to arise from the uterus. The mass also appeared to involve the right infundibulopelvic ligament with intense postcontrast enhancement and multiple areas of haemorrhage and necrosis. A small volume of ascites was noted. CT of the thorax, abdomen and pelvis (CT-TAP) was recommended. This did not demonstrate any pathologically enlarged lymph nodes. Of note, there was no evidence of disease in the vena cava or in the RA on preoperative imaging. Following multidisciplinary team discussion, preoperative differential diagnoses included degenerating leiomyomata and leiomyosarcoma. The patient progressed to complete staging surgery the following week.

Midline laparotomy was performed under general anaesthesia. On exploration of the abdomen and pelvis, a multilobulated mass arising from the uterus and distorting all pelvic anatomy (figures 1 and 2) was found. There were dense adhesions to the left pelvic side wall and bladder. The left ovary was adherent to the multilobulated mass. Engorgement of both infundibulopelvic ligaments was noted, with tumour appearing to invade the ovarian vessels. Bilateral salpingo-oophorectomy was completed given the extensive disease and concern for oestrogen-dependant pathological spread. Given the dense adhesions, size of specimen and difficulty mobilising, the infundibulopelvic ligament was ligated and divided approximately 3–4 cm proximal to ovary bilaterally. Pelvic lymphadenectomy of the left side was performed. Total operative time approached 5 hours. The measured blood loss was 2000 mL, which occurred during initial resection of the specimen. Due to appropriate anaesthetic care, the patient remained normotensive throughout the surgical resection, until removal of the hysterectomy specimen. At this point, the patient desaturated acutely and became profoundly hypotensive.

Figure 1.

Hysterectomy specimen—posteroinferior view.

Figure 2.

Hysterectomy specimen—anterosuperior view.

Differential diagnosis

The primary differential diagnosis for this acute deterioration was a large pulmonary embolism. Thrombosis within the pelvic vasculature due to extravascular compression by the uterine mass was suspected. This was thought to have embolised following manipulation of the pelvic vasculature at hysterectomy.

Intraoperative transoesophageal echocardiography (ECHO) was performed by the anaesthetic team. ECHO showed a large mass extending from the inferior vena cava (IVC) to the RA (figures 3 and 4). The mass was mobile and did not cause inlet or outlet obstruction of the RA. The patient’s saturations and blood pressure normalised, the midline laparotomy wound was closed and the patient was transferred to the intensive care unit under general anaesthesia. A multidisciplinary discussion was held to agree on the optimal management plan and this was then discussed with the patient’s next of kin.

Figure 3.

Echocardiogram image of atrial tumour extending from inferior vena cava.

Figure 4.

Echocardiogram image of mass in right atrium.

Treatment

Discussion between gynaecologists, cardiothoracic surgeons and anaesthetic staff immediately postoperatively concluded that this mass was a likely tumour embolism to the RA. This was suspected due to the intravascular extension of disease noted intraoperatively, and the robust, mobile and well-defined nature of the mass on ECHO. Although the patient had now stabilised, there was concern that this tumour could cause right atrial outlet obstruction. A collective decision was made to proceed directly to thoracotomy and retrieval of the atrial mass. The patient’s next of kin was counselled throughout this process and consent obtained. The patient was not extubated for discussion due to the anticipated risk of decompensation and emergent need for cardiothoracic retrieval of the embolus. Following midline sternotomy and opening of the RA, a worm-like fibrous tumour was noted in the RA with its tail extending into the IVC (figure 5). This mass was easily removed with tissue forceps from the RA (figure 6) and the sternotomy was closed.

Figure 5.

View of open right atrium at midline sternotomy with atrial tumour visible and arising from inferior vena cava.

Figure 6.

Right atrial tumour specimen.

Outcome and follow-up

The patient recovered very well postoperatively. On the second postoperative day, CT-TAP with venography showed no residual evidence of disease within the abdominal or pelvic vasculature. This negated the need for therapeutic anticoagulation. The patient recovered excellently and was discharged home on day 12 postoperatively.

Histological examination of the uterine mass showed uterine leiomyoma, with intravascular extension into the left infundibulopelvic ligament. Examination of the right atrial tumour also showed leiomyomatosis with a bland spindle cell tumour with foci of hydropic degeneration similar to hysterectomy specimen.

Thus, the diagnosis in this case was benign uterine leiomyoma with intravenous leiomyomatosis, and intraoperative intracardiac embolism of venous tumour following removal of the enlarged uterus at hysterectomy. Magnetic resonance venogram (MRV) at 6 months postoperatively did not demonstrate recurrent disease.

Discussion

Although intravenous leiomyomatosis with right atrial extension has been reported previously,8 11 12 17 18 this is the first reported case of intraoperative embolisation of tumour to the RA. We deduce that the cephalad aspect of intravenous tumour (figure 6), once transected from the main body of the pelvic mass (figures 1 and 2), embolised through the vena cava to the RA. In our case, comprehensive multidisciplinary assessment with our anaesthetic and cardiothoracic colleagues led to prompt diagnosis and management and stabilisation of the patient.

Intravenous leiomyomatosis can be extremely difficult to diagnose pre-operatively.^{10 17} Hints for diagnosis in this case were the atypical appearance of the leiomyomatous tumour on preoperative imaging. It is crucial that gynaecologists are cognizant of intravenous leiomyomatosis with its quasi-malignant behaviour,⁸ risk of recurrence¹⁵ and newly observed risk of intraoperative embolisation. In patients where preoperative imaging shows an unusual appearance of a leiomyomatous disease with potential intravenous extension, preoperative MRV should be considered. Thorough knowledge of vascular extension of disease from MRV will allow the surgeon to transect vessels proximal to disease. This will facilitate complete resection and prevent cardiac embolisation of tumour.

Patient’s perspective.

My journey took two and a half years from my first doctor’s visit until June 1st 2018 the day of my operation. Today I’m still in the process of healing and dealing with the aftermath of my illness.

It started for me in December 2015 with an awful pain in my womb after urinating. I went to the local doctor and was treated me for suspected urinary tract infection. I was given antibiotics and so I proceeded to take them over the course of a week. I was miserable over that Christmas and felt like the antibiotics weren’t working. I drank plenty of fluids and cranberry juice hoping it would help. By January I was feeling a little better and was going about my daily chores. However on a Monday in mid January 2016, I woke up feeling the need to urinate, but the urine wouldn’t come. I thought to myself that the urinary tract infection had come back and proceeded to drink water and try and start flushing my system. I went to bed to try and sleep, hoping that it would relax me. However the pain in my womb became unbearable along with the incredible urge to urinate. I got up to pee and to my horror I actually couldn’t urinate whatsoever. I became very distressed as my abdomen became distended and the urge to push as if I was in labour became excruciating. My husband rang for an ambulance and I was taken to the local hospital. I was given a urinary catheter which to my great relief helped me to urinate and immediately I felt better. The swelling in my tummy went down and the pain had gone. I remained in the Emergency Department with the catheter for a number of hours. Bloods were taken and it was revealed I had an infection. I was given a prescription and sent home.

Unfortunately this became my life for a number of months. I became distended and unable to urinate with great pain and was going back and forth to the doctor who prescribed me antibiotics for urinary tract infections causing urinary retention. Gradually the symptoms became worse. Eventually the pain got so bad I was unable to walk, drive or tackle everyday tasks. I was unable to make plans to travel anywhere and had to make sure that there was a toilet located nearby because the the insatiable urge to pee would be horrendous. I looked seven or eight months pregnant which interfered with my self-esteem and brought me to my lowest point in my life. Interestingly, I did notice a connection where the urinary retention coincided with my time of ovulation. The distension lasted 4 days in the beginning eventually leading up to seven days. I would immediately feel great relief when I began my menstrual period and my distended tummy became flat again. I also gradually noticed the distention was lasting longer and longer. The GP told me that it was part and parcel of hormones and being perimenopausal considering I was over forty, but I started to think that maybe something else was wrong. The pain had become unbearable. Yet again I went to the doctor and I was treated for urinary tract infection.

Then, in December of 2016, Then, I again went to the local GP Practice and met with a new General Practitioner who took one look at me and told me to go to the Emergency Department immediately, thinking it could be cancer. As it was Christmas week I said I would wait till after Christmas and go to the Emergency Department in January as I thought that if it was cancer, I was going to enjoy Christmas with my family. The new GP gave me antibiotics and asked me to come back and see her after Christmas. When I went back the first week in January and she saw the distention hadn’t gone down as much as she would have liked. She could also feel a lump in my stomach and told me to go to the Emergency Department. I did this the following day and was examined, had a scan and it revealed a mass. They couldn’t tell me what the mass was. They couldn’t tell if it was a fibroid or two fibroids going side by side. I had a second scan and it was discovered that it was one huge mass.

The doctors I met at my appointments in the early months of 2016 I were fantastic. I felt like for the first time I was being listened to to and I felt hope that the pain I was in was going to come to an end and I was going to get my life back. I was given an appointment for an MRI and CT scan and it was revealed that I had a mass the size of a 26 week foetus. I was given a course of tablets for three months to put me into false menopause to see if it would shrink the mass. Initially I felt some relief and for the first time in a long while I was able to walk, as I was previously unable to do my walking exercise due to my discomfort in my tummy. This gave me great hope and I thought I was on the right road to recovery. However after the three months of treatment were up, it felt that this had aggravated the mass and the suffering I went through became worse and I was even more distressed. I could feel the mass pulsating and this was greatly upsetting. When I went back for my appointment in April I was told that they had decided to refer me to a different consultant with more advanced gynaecological surgical experience, as the mass was too big to be dealt with in that clinic. I was referred and for the first time, I felt like I was going to get better. He listened to me and agreed to help me. I cannot fault my gynaecological surgeon and his team especially his specialist registrar. The care I received was amazing and I was put at ease which helped with my anxiety about what was going to happen. I was put on innohep and had to inject myself every day. It was decided to to perform a total hysterectomy which I didn’t really want but understood that it had to be done as there was no other choice. This upsets me but I knew realistically if I wanted to feel well it had to be done.

I went for an MRI scan and had my pre-op done and went ahead and had my operation. I was full of anxiety that day and was naturally upset. I have to say the staff in the theatre were brilliant and put me at ease especially my consultant. However I was a worried myself that maybe it wouldn’t be a straightforward as people thought.

When I woke up from my operation I was disorientated and confused as it was dark and I didn’t know where I was. I thought I would be in the recovery ward. Instead I had tubes coming from everywhere and was on a ventilator. I had a pain in my chest and I could not figure out why. A nurse explained to me that I was in the Intensive Care Unit. It took me a few minutes to digest what I was being told and and I was trying to figure out why I was there. After all, I only went in for a hysterectomy. The following morning a team of cardiothoracic surgeons explained what happened. I had suffered heart failure during the hysterectomy operation. It was discovered that I had a tumour in the heart. I was totally shocked and really couldn’t take it in what they were telling me. It wasn’t till I got home that I could grasp the enormity of what had happened and how lucky I actually was. I was indeed in the right place at the right time. The mass in my womb had sent a tumour up through my body and it found its way into the right side of my heart into the right chamber. I was a very lucky woman that day. I am very lucky to have had my surgeon and his wonderful team take care of me. If it wasnt for him and his team and their quick thinking I do believe I wouldn’t be here today. The care I received afterwards was amazing. I cannot fault the kindness I received from the doctors and nurses who are stretched beyond belief in this health system. I’m still on the road to recovery and have have had Magnetic Resonance Venography scans to ensure that nothing was left behind to start growing again. If it wasn’t for my gynaecological and cardiothoracic surgeons and their teams, I wouldn’t have got well enough to go on holiday to Australia this year, which I enjoyed so much. I thank everybody for giving me the opportunity to do that.

I do feel, however, that if the cause of my symptoms had been investigated more thoroughly during the early stages I wouldn’t be in the position I am in today. Earlier investigation and treatment may have prevented my condition from becoming so severe. I feel the major surgery I had may have been prevented if at local level, doctors listened closely to their patients and take seriously what women have to say. If they do, perhaps the next woman coming behind me will not suffer like I did, or go through the trauma of losing their womb.

Kindest Regards to everyone.

Learning points.

• Newly reported in this case, cardiac embolisation of leiomyomatous tissue during pelvic surgery is a potential sequelae of intravenous leiomyomatous disease. Where intravenous leiomyomatosis is suspected, care should be taken to identify the proximal aspect of intravascular tumour extension. Surgical technique should then aim to transect vasculature proximal to this disease. Thus, intraoperative embolisation of tumour and need for thoracotomy may be avoided.

• Uterine fibroids, although an extremely common gynaecological condition, can have a wide range of presentations with serious perioperative implications. Careful preoperative review of history, clinical examination and radiological images is essential in evaluating atypical uterine fibroids. Symptoms of both typical and atypical uterine fibroids may be insidious and difficult to piece together, especially in the absence of menorrhagia with Mirena coil, as in this patient.

• Where intracardiac leiomyomatosis is diagnosed preoperatively or embolisation occurs intraoperatively, resection of cardiac tumour may be performed immediately after laparotomy (one-step procedure, as in this case) or as a two-step procedure. We advocate immediate retrieval which in our case resulted in a prompt and full recovery for the patient.