A 32-year-old man, with a medical history notable only for severe hypertension, was referred to our hospital for hydrocephaly, secondary to a voluminous, hypervascularized, right tympanic tumour encasing the jugular vein and extending to the posterior fossa ( Fig. 1 A ).
On admission, the patient was dyspnoeic. Blood pressure was 180/93 mmHg and heart rate was 117 beats/min. Plasma brain natriuretic protein concentration was 2200 pg/mL. Echocardiography showed left ventricular (LV) dilatation (end-diastolic diameter 70 mm) and non-obstructive global LV hypertrophy (LV mass index 200 g/m 2 ). LV ejection fraction was 30%. These findings were confirmed by magnetic resonance imaging (MRI; Videos 1 and 2 ), which also demonstrated cardiac fibrosis ( Fig. 2 ). Because of the presence of both a tumour and hypertrophic cardiomyopathy, metaiodobenzylguanidine (MIBG) and octreotide scintigraphy were performed. These showed high fixation of the tumour, consistent with a paraganglioma ( Fig. 1 B). No other tumour foci were identified. Urine analysis confirmed catecholamine levels five to 10 times normal values and genetic testing demonstrated a characteristic mutation for paraganglioma (SDHC gene).
