British Journal of Radiology (2007) 80, e50-e53
© 2007 British Institute of Radiology
doi: 10.1259/bjr/94870835
Cardiac metastasis: a cause of recurrent pulmonary emboli
A D Borsaru, MBBS, RANZCR
1
K K Lau, MBBS, RANZCR
2 and
P Solin, MBBS, FRACP
3
1 Monash Medical Centre, 2 Diagnostic Imaging Department and 3 Department of Respiratory Medicine, 246 Clayton Road, Clayton, Melbourne, VIC, Australia
Correspondence: Dr Adina D Borsaru, Monash Medical Centre, 246 Clayton Road, Clayton, Melbourne, VIC, Australia. E-mail: adina_borsaru{at}hotmail.com
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Abstract
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Intracavitary cardiac metastasis is rare. Apart from causing intracardiac obstruction, this type of metastasis can present as pulmonary emboli. It is important to suspect this diagnosis in an oncology patient with recurrent pulmonary emboli, particularly in the setting of a negative venous Doppler ultrasound of the lower limbs and pelvis. Early recognition may help in improving the prognosis. We present a case of intracavitary cardiac metastasis arising from a squamous carcinoma of the cervix, in a patient with recurrent pulmonary tumour emboli.
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Introduction
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Intracardiac metastases, although rare, do occur in patients with widespread metastatic disease. They usually present as intracardiac obstruction; however, they can occasionally present as pulmonary emboli. It is important to suspect this diagnosis, particularly in the setting of a negative Doppler ultrasound for the lower limbs and pelvis, as early recognition and treatment may improve the prognosis. Here is a case of intracardiac metastasis arising from a squamous carcinoma of the cervix presenting clinically as recurrent pulmonary tumour emboli.
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Case report
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A 42-year-old female initially presented to another hospital with an acute episode of respiratory distress. She had been diagnosed with stage 4B squamous cell carcinoma of the cervix, with pelvic and para-aortic lymphadenopathy, approximately 6 months prior to presentation and had been treated with radiotherapy. She was examined on admission with a ventilation–perfusion scan (Figure 1a,b
) and a CT pulmonary angiogram, which were reported to have found emboli in several segmental branches of the right middle and lower lobe pulmonary arteries. She was anticoagulated with warfarin, improved clinically and was then discharged home.
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Figure 1. Ventilation–perfusion lung scan (left posterior oblique view) at the time of presentation shows several unmatched ventilation perfusion defects, consistent with pulmonary emboli.
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She re-presented with an episode of pleuritic chest pain 4 weeks later. The ventilation perfusion lung scan showed the presence of multiple unmatched ventilation–perfusion defects in both lower lobes, and the distribution was more extensive than those reported on the initial CT pulmonary angiogram. The appearances were consistent with recurrent pulmonary emboli (Figure 2). The patient's treatment was changed to Clexane, a low molecular heparin.
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Figure 2. Ventilation–perfusion lung scan at the time of the second presentation shows a marked increase in the number of the unmatched ventilation perfusion defects, consistent with recurrent pulmonary emboli.
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She then developed per rectal bleeding, disseminated intravascular coagulation syndrome and thrombocytopenia due to Clexane-induced heparin-induced thrombocytopenia syndrome (HITTS). The anticoagulation was ceased. A prophylactic inferior vena cava filter (Figure 3) was inserted, despite negative results of several lower limbs venous Doppler ultrasound studies. Unfortunately, the patient still deteriorated clinically with increasing shortness of breath and developed signs of right heart strain, suggestive of further episodes of pulmonary emboli. This prompted an echocardiogram, which revealed an intracardiac mass in the right ventricle, causing an outflow obstruction and right atrial dilatation (Figure 4a,b). A contrast enhanced CT scan of the chest, abdomen and pelvis was subsequently performed and confirmed the presence of the intracardiac mass associated with a small pericardial effusion (Figure 5a,b). The CT scan did not show any pulmonary metastasis, inferior vena cava or pelvic vein thrombosis.
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Figure 3. Transthoracic echocardiogram showing a large mass lesion arising from the right ventricle and extending across the tricuspid valve into the right atrium.
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Figure 4. Post-intravenous contrast axial CT scan through the mediastinum shows a large pulmonary embolus in the distal portion of the right main pulmonary artery.
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Figure 5. Post-contrast axial CT scan through the thorax shows a large irregular filling defect in the right ventricle, extending to the right atrium through the tricuspid valve.
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In view of the outflow obstruction caused by the right ventricular mass, the patient underwent an open excision of the intracardiac mass (Figure 6). At surgery, the mass was seen arising from the free wall of the right atrium, extending through the tricuspid valve into the right ventricle. It occupied 90% of the right ventricular lumen resulting in right heart outflow obstruction and, at the same time, invaded the cordae tendinae. The histopathology of the surgical specimen revealed this to be an intracardiac metastatic deposit from cervical squamous cell carcinoma (Figure 7). The intracardiac metastasis was believed to be the cause of the recurrent pulmonary embolic events that she had suffered.
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Figure 7. Histopathologically the mass consists of poorly differentiated anaplastic cells with keratinization and intercellular bridge formation consistent with metastasis arising from a squamous cell carcinoma of the uterine cervix.
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Discussion
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Metastatic disease to the heart is rare. The incidence quoted in the current literature, based on autopsy, is approximately 1.23% [1]. The low incidence of cardiac metastasis is classically attributed to a combination of factors: the continuous myocardial contraction, the metabolic particularities of the striated cardiac muscle, the rapid flow of blood through the heart and the lymph flow away from the heart [2]. They remain, however, 20–40 times more common than the primary cardiac tumours, accounting for approximately 96.5% of all cardiac tumours. Although they are rarely diagnosed during lifetime, their incidence rises to 10–20% in autopsies of patients with known malignancies [3, 4].
Approximately three-quarters of primary tumours are carcinomas, followed by haematological malignancies, melanomas and sarcomas [5]. In the carcinoma category, the most common primary is lung carcinoma, especially adenocarcinoma, accounting for almost 36% of the cases. This is followed by breast carcinoma, comprising approximately 7%, and squamous cell carcinoma of the oesophagus. This is due to both their high incidence and their close proximity to the heart and mediastinum [3, 5]. Cardiac metastases from pelvic malignancies are rare with only a few cases reported in the literature. Ovarian tumours, including some with low malignant potential, uterine leiomyosarcoma, intravenous leiomyomatosis, choriocarcinoma, endometrial carcinoma and squamous carcinomas of the cervix have been implicated. To date, and to the best of our knowledge, there are only 14 cases of cardiac metastases from squamous cell carcinoma of the cervix reported in the literature [6].
Classically, there are four pathways of cardiac involvement: (1) retrograde lymphatic spread, (2) direct extension from the adjacent viscera, (3) haematogenous spread, and (4) transvenous extension through the vena cava into the right sided chambers.
The most common site of cardiac involvement is the pericardium/epicardium (in 33–37% of cases). This is commonly associated with a pericardial effusion (in 33.7% of cases), which is usually haemorrhagic [4]. The pericardial involvement is usually the result of lymphatic or direct spread, and is found most commonly in lung, breast and haematological malignancies [5, 7]. Myocardial involvement is much less common. It is usually the result of haematogenous spread, and it is associated with widespread disseminated disease, in particular lung metastasis. It is most common in melanoma and haematological malignancies. Intracavitary, endocardial or valvular metastatic deposits, such as the one described in our case, occur in less than 6% of cases [5]. Interestingly, approximately 80% of this type of metastasis occurs in the right chambers and only rarely in the left chambers. This is attributed to the filtering role of the pulmonary circulation and the slower flow in the right chambers [8].
Cardiac metastases are difficult to diagnose clinically and may go unrecognized until autopsy. The clinical presentation may include non-specific symptoms such as chest pain, weight loss, malaise, or more characteristic symptoms, such as congestive cardiac failure secondary to intracardiac obstructions, valvular involvement, or pericardial effusions; arrhythmias due to involvement of the conduction system, or, as in our case, embolic events. Thus, metastases should be suspected in oncology patients if they develop inexplicable heart failure, neurological deficits or recurrent pulmonary emboli, particularly when no peripheral source for the emboli can be identified.
Methods of diagnosis include plain radiographs, transthoracic or transoesophageal echocardiography, cardiac angiography, CT and MRI. The plain chest radiograph is abnormal in almost 80% of the cases, but the findings are non-specific and may include an abnormal or enlarged cardiac contour, pulmonary oedema, or an enlarged azygous vein [9].
The echocardiography remains the most efficient method for the initial diagnosis. Transoesophageal echo has a better visualization of the atrias and the great vessels than CT, MRI and angiography. There was a case of percutaneous transoesophageal echo guided biopsy of a right atrial melanoma described in the literature [9–11].
The CT and MRI have become useful tools in imaging cardiac tumours. They add useful information regarding the tumour location, its morphological features, its extent, and the presence of local invasion and associated mediastinal and/or pulmonary involvement. They can also offer some degree of histological characterization of the tumour by the identification of fat, calcifications, fibrous tissue, melanin, haemorrhage or cystic changes. Administration of contrast in MRI may also help differentiating between tumour and bland thrombus, as tumours usually enhance. In addition, the pathophysiological effect of the tumour on the myocardial haemodynamics can be assessed by using dynamic imaging techniques in MRI [3].
Angiography helps to localize the tumour and to assess the valvular function, the anatomy of the coronary arteries and the great vessels in relation to the tumour, as well as to define the tumour vascular supply. This is important particularly when surgery is contemplated.
The treatment is generally palliative, aimed at reducing the obstruction to the blood flow by the tumour and to provide symptomatic relief. The options remain very limited and depend on the type of primary tumour and its response to chemotherapy or radiotherapy, the extent of intracardiac and extracardiac involvement, and the general status of the patient. They include open surgical excision, chemotherapy, or radiotherapy. Irrespective of the treatment, the prognosis remains very poor.
In conclusion, intracavitary tumour metastases from squamous cell carcinoma of the cervix are extremely rare. In oncology patients with recurrent pulmonary embolic events or unexplained cardiac symptoms, cardiac metastases should be suspected, particularly when a clear source for the emboli is not identified in the lower limbs.
Received for publication August 12, 2005.
Revision received February 8, 2006.
Accepted for publication March 20, 2006.
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References
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Abstract
Introduction
