Extra-adrenal Paraganglioma

Case Study: Extra-adrenal Paraganglioma

This 32 yr old male was admitted to hospital with acute SOB and bleeding from the nose. The patient also noted the veins in his neck were more prominent and he had a feeling of “head congestion”. No weight loss, night sweats or lymphadenopathy.

Initial echocardiogram (Figure 1) demonstrated a large pericardial effusion with some suggestion of tamponade. A large mixed echogenic “mass” (red arrows) measuring 8cm x 4.7 cm was shown within/compressing the left atrium. A percutaneous drain was inserted and 1700 ml of blood stained fluid was extracted from the effusion.

A CT scan of the chest and abdomen established the large mediastinal mass (Figure 2) to be quite vascular in the subcarinal region and not invading the left atrium or surrounding structures. A CT of the heart using a coronary angiogram protocol was also performed but found the coronary arteries unremarkable. An MRI with T1 and T2 weighted scans demonstrated multiple vessels around the mass due to collateral formation.

As part of  his assessment this man underwent a cardiac catheter investigation. An injection into the descending aorta demonstrated a large bronchial vessel supplying part of the tumour. Selectively catheterised with a Left 4 Judkins it outlined the left bronchial wall and typical of a bronchial artery but supply to the right side was large and this also supplied a sizable upper part of the tumour (Figure 3).

The right coronary artery had a very large vessel at the level of the right ventricaular branch, which ran posterior and superior to supply a large vascularity to the tumour (Figures 4).

The circumflex supplied two large vessels into the tumour arising from a branch at the level of the obtuse marginal and running posteriorly over the atrium (Figure 5).

The patient was screened for urinary catecholamines and vanillylmandelic acid as part of the investigation and this came back positive. A MIBG scan was then undertaken and  demonstrated an area of increased uptake in the region of the mediastinal mass, showing features consistent with that of an extra-adrenal paraganglioma/phaeochromocytoma.

Following selective catheterisation and embolisation of the collateral branches, a follow-up CT of the chest was performed with contrast that demonstrated a reduction in vascularity.

The patient is currently awaiting surgery.


Figure 1

Figure 1

Figure 2

Figure 2

Figure 3

Figure 3

Figure 4a

Figure 4a

Figure 4b

Figure 4b

Figure 5

Figure 5


Author:

Mr Mark Bowers
Superintendent Radiographer
Harefield Hospital
UK

 

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