Elsevier

Cardiovascular Pathology

Volume 19, Issue 6, November–December 2010, Pages 343-352
Cardiovascular Pathology

Original Article
Pathological study of primary cardiac and pericardial tumours in a specialist UK Centre: surgical and autopsy series

https://doi.org/10.1016/j.carpath.2009.07.005Get rights and content

Abstract

Background

Primary cardiac and pericardial tumours are rare with a prevalence of between 0.001% and 0.3%. Thus, general pathologists are not familiar with them. Modern advances in cardiac imaging have increased the number of patients identified with a primary cardiac tumour in its early stage and also improved prognosis. At the Royal Brompton Hospital, London, we did a retrospective study to investigate the pathological features of primary cardiac and pericardial tumours and compared our findings to other cardiac centres.

Methods

All pathologic records at the Royal Brompton Hospital between 1990 and 2008 were reviewed to identify patients with a confirmed diagnosis of primary cardiac tumours. A total of 94 patients with a histological diagnosis of primary cardiac and pericardial tumours were identified and formed the study population.

Results

The majority (n=67, 71.3%) of cases were benign cardiac tumours. Myxoma was the most common histologic type accounting for 27 cases. Among cases with primary malignant tumours (n=27, 28.7%), unclassified sarcoma (n=11), leiomyosarcoma (n=5), and lymphoma (n=4) were the most common histologic types.

Conclusion

This study, primarily from an adult setting (n=78, 83%) demonstrates a large spectrum of cardiac tumours seen in recent cardiologic practice. Myxoma is still the most common tumour but more fibroelastomas are being diagnosed due to increased imaging.

Introduction

Primary tumours of the heart are rare and were considered for a long time a post-mortem diagnostic curiosity. Although uncommon, cardiac neoplasms represent an important cause of morbidity and mortality in clinical cardiology. Their potentially lethal course and the possibility of cure with excision make their diagnosis challenging and consequential. Therefore, clinicians and pathologists need to be aware of cardiac tumour pathology as well as their frequently atypical clinical presentations. Antemortem recognition of intracardiac tumours by clinical and electrocardiographic findings was first recognized in 1934. In 1951, the diagnosis of an intracavitary left atrial tumour was confirmed with angiography and the first successful excision of a heart tumour, a left atrial myxoma, was reported in 1955. It was only later in the 1970s when M-mode and 2D echocardiography became available together with other imaging techniques such as computed tomography and magnetic resonance imaging (MRI), that antemortem diagnosis of intracardiac tumours became more feasible [1].

Based upon the data of 22 large autopsy series, the frequency of primary cardiac tumours was 0.02%—corresponding to 200 tumours in 1 million autopsies making them very rare indeed [2]. An increase in the incidence of primary cardiac tumours has been reported since the development of non-invasive cardiac imaging modalities. These imaging techniques have highlighted larger surgical series. Among 27,640 patients assessed for cardiac disease, the incidence of tumours was 0.06% (1980–1984), 0.22% (1985–1989), and 0.32% (1990–1995), reflecting the higher detection of these tumours during life [3]. With this increased detection, general pathologists need to be aware of their pathology. Also since patients may present in atypical ways such as weight loss, diarrhoea, muscle aches, sudden death [4], pulmonary or systemic emboli [5], or acute myocardial infarction [6], pathologists may encounter them in unexpected clinical and autopsy situations. Here at a specialist referral centre, we have reviewed all our primary cardiac and pericardial tumours over the past 18 years to show the main types and compare them with other series from USA and Europe.

Section snippets

Methods

All pathologic records at the Royal Brompton Hospital, London, between 1990 and 2008 were reviewed to identify patients with a confirmed diagnosis of primary cardiac tumours. A total of 94 patients with a histological diagnosis of a primary cardiac and pericardial tumour were identified and formed the study population. Both surgical and autopsy specimens were included. The tumours were seen by a number of pathologists, particularly as many cases were referrals.

We obtained ethical consent to

Results

We identified 94 primary cardiac and pericardial tumours, spanning the years 1990–2008. Of the 94 cases, 67 (71.3%) were primary benign tumours, and 27 (28.7%) were primary malignant tumours (Table 1). Of the 67 benign cases, there were 27 myxomas, nine benign mesothelial cysts, eight papillary fibroelastomas, four fibromas, four inflammatory myofibroblastic tumours, three lipomas, three teratomas, three paragangliomas, two rhabdomyomas, two haemangiomas and two atrioventricular (A-V) nodal

Discussion

This series reflects many of the previous series of primary cardiac tumours, but there are important differences and evolving concepts with these rare tumours. Primary tumours of the heart and pericardium are rare with an incidence between 0.0017% and 0.028% in collective series [7]. In the US, based upon the data of 22 large autopsy series, the prevalence of primary cardiac tumours is approximately 0.02% (200 tumours per million autopsies). About 75% of primary tumours are benign, and 50% of

Conclusion

Primary cardiac tumours represent an important cause of morbidity and mortality in clinical cardiology that affects patients of all ages. Most primary cardiac neoplasms are benign, which are curable with surgery, and therefore, early diagnosis and resection are possible. They are more likely to occur on the left side of the heart with right sided tumours more likely to be malignant. This study demonstrates a large spectrum of cardiac and pericardial tumours seen in recent cardiologic practice

Acknowledgments

We are very grateful for a grant from Cardiac Risk in the Young, UK, which has enabled us to carry out this work.

CRY, Unit 7, Epsom Downs Metro Centre, Waterfield, Tadworth, Surrey, KT20 5LR. [email protected].

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    Funding has been acknowledged by the Royal Brompton NHS hospital trust. Both authors have NO commercial association or other arrangement (financial compensation, potential to profit, consultancy, stock ownership, honor, patent-licensing arrangements, etc) that might pose or imply a conflict of interest in connection with the submitted paper.

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