You have been asked to see a 56-year-old male inpatient who presented 2 months previously with progressive shortness of breath and fever. He was discovered to have a large pericardial effusion which was tapped and was found to contain poorly differentiated cells. Subsequent CT imaging showed him to have widespread pulmonary metastasis with a 3 cm lobulated mass arising from and projecting into the right atrium, compressing the inferior vena cava. Biopsy tissue was obtained and confirms angiosarcoma.
Which one of the following statements is true?
The most common of the primary cardiac malignant tumours, angiosarcoma is a tumour of endothelial cell differentiation. It is found in patients of all ages, with a peak in the fourth decade, and there is no sex predilection. The most common site is the right atrium. Angiosarcoma of the heart is considered primary if there is no evidence of previous or concomitant tumours in the soft tissue, bone, or subcutaneous tissue. Presenting symptoms include fever, myalgia, and weight loss as well as chest pain and arrhythmias. In more advanced disease, lung metastasis, congestive heart failure, and large pericardial effusions can be seen. Endomyocardial biopsy can provide in vivo diagnosis. Echocardiography will demonstrate an irregular echogenic mass, typically associated with a pericardial effusion. Angiographically the mass can appear to be highly vascular. CT and CMR show the heterogeneity of the mass, including tissue necrosis and haemorrhage. One-third of angiosarcomas are poorly differentiated, and two-thirds are moderately differentiated. Cells can express endothelial cell antigen (factor VIII, von Willebrand factor, CD31, and CD34), and mutations of the TP53 and K-ras genes have been reported. Mean survival is 10 months.
A 47-year-old female with known metastatic breast carcinoma has been admitted to the oncology ward for investigation of increasing shortness of breath. A pulmonary embolism was suspected on clinical grounds and a CT pulmonary angiogram was performed. It failed to demonstrate any pulmonary emboli but did show a 2.2 cm global pericardial effusion. On the basis of this, a transthoracic echocardiogram was performed which ruled out tamponade.
Which one of the following statements is correct?
Cardiac metastases occur via four routes:
Pericardial metastases typically arise from lymphatic spread whereas haematogenous spread preferentially gives rise to myocardial metastases. Endocardial tumour deposits are rarely found.
Owing to their location and prevalence, lung and breast carcinomas are the most common tumours causing cardiac metastases and both preferentially affect the pericardium, resulting in usually large effusions. Cardiac metastases are seen in around half the cases of metastatic melanoma.
Extracardiac tumours may reach the atria and ventricles by transvenous extension. Renal cell carcinoma growth through the inferior vena cava into the right atrium is thought to occur in up to 1% of cases. Rarely, bronchial carcinoma spreads through the pulmonary veins into the left-side heart cavities.
In cases of pericardial effusion causing circulatory embarrassment, pericardiocentesis is mandatory and may help diagnosis. Following pericardiocentesis, a constrictive picture often persists due to inflammation and thickening of the pericardium.
Regarding the prevalence of cardiac tumours:
Cardiac tumours have a prevalence rate of 0.056% for primaries and 1.23% for secondaries, with an approximate ratio of secondary to primary of 20:1 (Lam KY). The University of Padua has reported on an extensive post mortem series, which described 210 primary cardiac tumours, of which 89% were benign and 11% malignant. In this series, papillary fibroelastoma was the second most common benign primary cardiac tumour after atrial myxoma (Basso C).
Lung carcinoma is the most common metastatic cardiac tumour (approximately 33%). Lymphoma and leukaemia (16%) are the second most common followed by breast (5%), hepatic (5%), and kidney (4%) carcinomas.
Atrial myxoma is a benign disease that does not metastasize. It typically presents with constitutional, obstructive, or embolic symptoms. In the primary benign cardiac tumours, there is a female predominance of 1.4:1. The mean age is 47 years.
A 57-year-old male patient is discovered to have an echogenic mass arising from the insertion point of the anterior mitral valve leaflet. He undergoes uneventful surgical resection. The mass is described as reddish and nodular. Frequent areas of calcification are identified by naked eye. Histologically, it is composed of oval CD34, CD31, and factor VIII positive cells consistent with epithelioid haemangioendothelioma.
Which one of the following five statements regarding this tumour is true?
Previously considered a low-grade or borderline malignant vascular lesion, epitheloid haemangioendothelioma is classified as a malignant tumour, along with angiosarcoma, because of its local aggressiveness and metastasizing potential. It is a very rare tumour.
There is a spectrum of disease with benign epithelioid haemangiomas at one end and the highly malignant epithelioid angiosarcoma at the other. The epithelioid haemangioendothelioma sits in the middle. Systemic metastasis is reported in approximately 20% of cases described in the medical literature (Lisy M). Treatment is radical surgical resection. There is limited value in radiotherapy and chemotherapy (Moulai N).
Originating from the subendocardium, these tumours can occur at any location within the heart. The expression of vascular endothelial markers, such as von Willebrand factor, CD31, and CD34, rules out metastatic adenocarcinoma or melanoma. The prognosis is unpredictable and typically poor
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