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Extensive pulmonary metastases in a young boy with primary cardiac angiosarcoma

Gerhard Van Wyk

Malignant primary cardiac neoplasms are rare and of them, sarcoma (75%-95%) and specifically primary cardiac angiosarcoma (30- 40%) is the most common subtype 1–5. These tumours are known to be aggressive in nature and resistant to chemo- and radiotherapy and are associated with a poor prognosis due to delay in diagnosis and lack of directed therapies.

Clinical presentation depends on tumour location and size, and may include dyspnoea, fatigue, cardiac failure, atypical chest pain, and manifestations of metastatic disease. Tissue sampling for a histological diagnosis can be very challenging but is imperative for formulating a definitive therapeutic plan.

We present an unusual case of primary cardiac angiosarcoma in a very young, male patient characterised by a challenging diagnostic process and rapid disease progression following clinical presentation. This case also highlights the potential role of transvenous imaging- guided endomyocardial biopsy as a method to obtain tissue samples where conventional methods may not be feasible.