I would like to commend Kelsall’s1 initiative to disseminate knowledge on rare diseases. The initiative will certainly help identify, stimulate, coordinate and support research, which could benefit patients who have any one of the nearly 6800 known rare diseases.
I would like to draw attention to tumours of the heart. Primary cardiac tumours represent only 25% of all cardiac tumours. The most prevalent are metastatic cardiac tumours and pseudotumors. Estimated frequency of primary tumours of the heart ranges from 0.0017% to 0.33%.2 Seventy-five percent of primary cardiac tumours are benign. Myxomas account for nearly half of them. Primary malignant cardiac tumours are predominantly sarcomas.2
Since noninvasive diagnostic modalities have become more sensitive, there has been marked increase in the number of patients who receive diagnoses.
Presenting symptoms, treatment options and prognosis are largely controlled by the anatomic location of the tumour. Tumours of the heart are known to be great mimickers.2,3 Most patients with malignant cardiac tumour die within one year of initial diagnosis, either because of the often asymptomatic presentation of cardiac tumours until advanced disease or because of a low index of suspicion on the part of the physician. The survival rates for extracardiac malignancies have improved substantially. Meanwhile, the prognosis in cardiac malignancies is still dismal. Median survival for patients with cardiac sarcomas is 6 months, and 93 months for those with noncardiac sarcomas. Patients with primary cardiac sarcomas who undergo surgical resection have a median survival of 12 months. Patients who undergo only palliative chemotherapy have a median survival of one month.4
Management options for cardiac tumours include surgery, neoadjuvant and adjuvant chemotherapy. Surgical resection is the treatment of choice.5 In the case of inoperable disease (i.e., unresectable tumour, presence of metastases) palliative chemotherapy should be offered, although in some cases, palliative surgical debulking may be undertaken to relieve rapidly progressing symptoms. Younger patients with no metastatic disease may be considered suitable candidates for orthotopic heart transplant.4