Eur J Cardiothorac Surg 2003;24:843-846
© 2003 Elsevier Science NL
Surgical clearance of invasive cardiac leiomyosarcoma with concomitant pneumonectomy
Betsy J. Evans*,
Marcus P. Haw
Wessex Cardiac Unit, Southampton General Hospital, Southampton, UK
Received 11 February 2003;
received in revised form 4 June 2003;
accepted 15 June 2003.
* Corresponding author. NHLI Imperial College, Hammersmith Hospital, Du Cane Road, W12 0NN, UK. Tel.: +44-7884-184-839; fax: +44-20-8383-1640
e-mail: b.evans{at}imperial.ac.uk
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Abstract
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The management of cardiac leiomyosarcoma is still controversial. Due to the rare occurrence and late presentation of such tumours the treatment is essentially palliative. We report on a case in a young female who presented with catastrophic haemodynamic instability and pulmonary venous obstruction by a large mass in her left atrium. Right pneumonectomy was performed as part of emergency surgery in this patient with a left atrial tumour of unknown histology. We feel that an attempt at complete clearance of these tumours is justified, as surgery remains the only chance of long-term survival. Postoperatively, adjuvant chemotherapy and/or radiotherapy may have a role.
Key Words: Cardiac leiomyosarcoma • Surgical clearance
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1. Introduction
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Cardiac leiomyosarcoma is a very rare tumour with a poor prognostic outcome [1,2]. The tumour is usually highly malignant and invades local tissues extensively often making it difficult to achieve surgical clearance. Leiomyosarcomas originating in the pulmonary vein are very rare [3]. Shimono et al. reported a case of one such tumour requiring left atrial wall resection combined with pneumonectomy via thoracotomy [4,5]. The largest worldwide series concerning primary left atrial leiomyosarcoma is that of the Armed Forces Institute of Pathology (AFIP), consisting of 12 cases, six of which were surgically resected [6]. Our case report demonstrates a primary cardiac tumour that originates in the left atrium with invasion and complete occlusion of the right pulmonary veins. Complete resection was made via median sternotomy with right pneumonectomy and subsequent reconstruction of the atrial wall and septum. Adjuvant chemotherapy followed surgery, and at 1 year follow-up the patient is well with no evidence of local recurrence.
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2. Case report
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A 41-year-old lady was admitted under the respiratory team on March 9 2002 for a bronchoscopy and open lung biopsy following a 6-month history of breathlessness, non-productive cough, lethargy and weight loss. Previous medical history was unremarkable. On examination there was no evidence of cyanosis, pulse and blood pressure recordings were normal, chest auscultation revealed clear lung fields with no additional heart sounds or murmurs detected. Chest radiograph showed fine interstitial infiltrate on the right side and an ECG was normal. Thoracic CT revealed changes in the lung parenchyma consistent with pulmonary veno-occlusive disease, however, biopsy did not provide a diagnosis. Following the procedure the patient had a respiratory arrest requiring re-intubation and full ventilatory support. Her clinical condition deteriorated because of increasing haemodynamic instability. Subsequent trans-oesophageal echocardiogram revealed a large mass within the left atrium occluding the right-sided pulmonary veins. Surgical excision of this undiagnosed mass was performed as an emergent case on March 14 2002. It was predicted that pneumonectomy may be required and consent was obtained from her husband.
Total cardiopulmonary bypass was instituted with bicaval and ascending aortic cannulation. Cardioplegic arrest was achieved with cold blood cardioplegia and the circulation cooled to 25 °C. The left atrium was opened at the base of the left atrial appendage a large fixed tumour was seen, which appeared to be malignant (Fig. 1
a,b). It was protruding from the body of the left atrium obliterating the right-sided pulmonary veins and within 1 cm of the left-sided pulmonary veins. A frozen section was not performed to avoid any delay in the procedure due to the fact the aortic had been cross-clamped, in addition resection was the only option if haemodynamic stability was to be restored. The aorta and pulmonary artery were transected in order to gain access to the roof of the left atrium and the whole of the left atrial wall was excised with the tumour en-bloc by circumnavigating its attachment. This left only the orifices of the left pulmonary veins, the left atrial appendage and a small patch of left atrium around the mitral annulus. The tumour was mobilized back to the hilum of the lung as much as possible, however, at this stage it became clear that to obtain complete tumour clearance a right pneumonectomy was necessary. A large patch of bovine pericardium was used to reconstruct the left atrium, atrial septum and right atrium. The patient was weaned from cardiopulmonary bypass without difficulty after normothermia and great attention was applied to obtain complete haemostasis.
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Fig. 1. (a,b) Photograph and line drawing: View through transected aorta (Ao) and pulmonary artery (PA) with roof of left atrium excised showing extensive tumour (T) protruding from the body of the left atrium obliterating the right-sided pulmonary veins (RPV) and with in 1 cm of the left-sided pulmonary veins.
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Histological examination of the resected tumour revealed it to be attached to the endothelial surface of the atrium, not infiltrating the wall. It had a variable growth pattern with areas of hyaline acellular collagen and there was a single focus of osteoid formation. This appearance was consistent with a high-grade sarcoma with focal osteoid formation and expression of muscle markers, best regarded as a leiomyosarcoma. The excision margins of the surgical specimen were clear of disease. The lung showed areas of interstitial fibrosis with marked thickening of the alveolar walls. There was marked intimal thickening of the large pulmonary veins, consistent with veno-occlusive disease. No evidence of malignancy was seen in the lung or lymph nodes.
This lady had a predictably stormy post-operative course due to ongoing pneumonia in the remaining lung. She was noted to have right upper limb weakness with a CT brain confirming watershed infarcts of the left occipital and left temporal lobes. She made a full functional recovery from these and was discharged home on April 12 2002. She received four cycles of ifosfamide and doxorubicin chemotherapy, which finished in July 2002. At1-year post surgery she is well with no evidence of any local recurrence. At her most recent follow-up she had suffered from blurred vision and unsteadiness. A CT brain revealed a presumed cerebral secondary.
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3. Comment
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Primary leiomyosarcomas are rare tumours, incidence being approximately 0.25% of all cardiac tumours; they are most commonly discovered in post-mortem examination. Consequently there does not currently exist a large series of reported cases and consensus on management is somewhat anecdotal.
These tumours are highly aggressive with a mean survival of 6 months [7]. They invade locally to give obstructive symptoms, usually of valves, or great vessels. Surgery is essentially palliative with the aim being to relieve obstruction, however, complete resection has been shown to alter mean survival time from diagnosis [2]. Most patients die as a result of metastases. Prognostic factors are difficult to define. A statistical difference in survival has been seen between cardiac sarcomas with less than five mitoses per 10 hpf compared with those with greater than five [1]. Other reported factors affecting survival are the presence of necrosis, location of the tumour on the right side of the heart, presence of metastases [8].
The adjuvant use of chemotherapy is the preferred post surgical treatment modality based on the high risk of both local and distant recurrence, reports exist demonstrating benefit with this type of tumour. Some authors combine the use of adjuvant chemo and radiotherapy post operatively and claim an increase in survival time, however, all of these reports are on small case numbers [9].
Our case demonstrates the classical late presentation of cardiac leiomyosarcoma with symptoms secondary to obstruction of pulmonary veins due to large tumour mass. The histological findings are also consistent with previous reported cases reflecting a high-grade tumour. The importance of echo in diagnosing this condition is also stressed since this patients symptomatology initially led to investigations on the pulmonary system, which all proved to be inconclusive [10]. The surgical dilemma is whether or not to perform concomitant pneumonectomy rather than deeming the tumour inoperable in an otherwise healthy young adult. We feel that this radical surgical approach was justified given that the literature and clinical judgement suggest it to be the only means of improving survival (see Table 1).
Clear excision margins were seen on our histology report, and no pulmonary or lymph node deposits of tumour were seen, however, it was decided to offer our patient postoperative chemotherapy based on the knowledge that the cause of death is most commonly due to metastases and that early chemotherapy has been shown to increase survival time.
Despite the rare incidence of primary malignant cardiac tumours the diagnosis should be considered in a case such as this where extensive pulmonary investigations were inconclusive in a previously well individual with no past history of lung disease. A consensus on the best medical practice for these patients will be difficult to obtain due to the rarity of the condition, however, reports on outcome following surgery and chemotherapy from different centres may be collated and analysed to gain more information for the future.
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References
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Abstract
1. Introduction
