Background: Primary right ventricular sarcoma is a very rare and fatal condition. The incidence of primary cardiac tumour is about 0.17-0.19%. Primary cardiac sarcomas associated with pulmonary hypoperfusion should be resected and radiation could alleviate the reduced pulmonary perfusion.

Methods: We reported a case of primary right ventricular sarcoma in a young patient who presented with persistent left sided pleuritic chest pain, fever and constitutional symptoms for 6 months. Initial tests showed a normal ECG and blood investigations. He was admitted to our ward for further cardiac investigations, including to rule out Acute Coronary Syndrome. Cardiac enzymes were normal but patient was still having persistent symptoms.

Results: His ECHO demonstrated a huge right ventricular mass occupying 80% of the right ventricle. The mass is lobulated and obstructing the right ventricular outflow causing severe reduction of blood flow to the pulmonary artery, causing pulmonary hypertension and subsequently cardiac failure. We proceeded with a cardiac MRI to study the nature of the lesion. After a MDT meeting with different teams, we decided to biopsy the mass instead of resection as operation poses a high risk to the patient. The biopsy came out to be a primary sarcoma. He was subjected to radiotherapy to reduce the size of the mass and planned for total resection by the cardiothoracic team later.

Conclusion: Primary right ventricular sarcoma is a very rare tumour. Radical surgical excision is the mainstay of treatment, but despite surgical resection, its prognosis is poor due to delayed diagnosis and early metastasis.