[Cardiac manifestations of peripheral primitive neuroectodermal tumor (pPNET): a rare case]

A 44-year-old man presented to his general practitioner with increasing exertional dyspnoea. After a syncope he was admitted to hospital.

Echocardiography on admission demonstrated a large pericardial effusion (45 mm over the right and 57 mm over the left ventricle) which, in view of its haemodynamic relevance, had to be removed by pericardiocentesis. Cytological examination of the fluid showed various blood constituents but no atypical cells. Computed tomography (CT) of the thorax revealed a homogeneous hyperdense structure in the area of the left atrium. After several more pericardiocenteses echocardiography showed a pericardial tumour between the left atrium and ventricle. Gastroscopy, coloscopy, abdominal CT, an octreotide scan and positron-emission tomography did not indicate an extracardiac tumour. Coronary angiography and video-assisted thoracoscopy were performed. They confirmed an epicardial tumour at the level of the left atrium.

The entire round and solid tumour of about 3 cm in diameter, at the tip of the left atrial appendage was removed under extracorporeal circulation. No adjuvant treatment was given. Histological and immunohistological tests of the resected specimen (CD-99 and NSE-positive) provided the diagnosis of malignant peripheral primitive neuroectodermal tumour (pPNET).

There are at present no adequate data on optimal treatment of pPNET in the heart. This case demonstrates that even with a large such tumour long remission is possible after complete removal without any adjuvant treatment.