CASE REPORT Multiple phaeochromocy- toma (adrenal and extra-adrenal) L D R Tsatsi MS ChS, FCRad(D}SA M A Mabiletsa SSe, MSChS Department of Diagnostic Radiology Medical University of Southern Africa Case presentation A 17-year-old-girl was referred from a rural hospital with problems of hypertension secondary to suspected coarctation of the aorta, and aortic incompetence. Her main complaints were headaches and palpitations. Clinicallyshe had mild to moderate hypertension, tachycardia, left ventric- ular enlargement and a systolic mur- mur. There were no abdominal masses. Investigations performed excluded coarctation of the aorta and aortic incompetence. ECG confirmed sinus tachycardia with left ventricular hypertrophy. Renal artery stenosis was also excluded by a normal renogram. She was then investigated for other causes of hypertension including phaeochromocytoma. Serum urea and electrolytes were normal. Urine vanillylmandelic acid (VMA) and metanephrines were also normal. Imaging CXR There were no positive findings despite clinical early cardiomegaly. MIBGscan This showed no features of phaeo- chromocytoma. Ultrasound A homogeneous soft-tissue mass measuring 4.5 x 4.2 em, slightly lobu- lated and well defined was visualised on the left side of the aorta just above its bifurcation. No calcifications were noted. The aorta was not compressed. Another similar mass measuring 2.9 x 2.9 em, triangular, and more reg- ular in outline, was visualised at the upper pole of the left kidney. There were no other positive find- ings (Fig. I). CT scan of the abdomen The patient was allergic to iodine and had to be premedicated with prednisone, and alpha-blockers were also given to avert a hypertension crisis (Fig. 2). A homogeneous retroperitoneal tumour, isodense to skeletal muscle, was found to the left of the aorta just above its bifurcation. It was lateral to the aorta, and anterolateral to the spinal column and left psoas muscle. It measured 6.0 x 4.2 x 5.8 em. The 22 SA JOURNAL OF RADIOLOGY. September 2002 Fig. 1. Transverse sonar mid-abdomen showing left para-aortic mass - organ of Zuckerkandl. Fig. 2. Pre-contrast CT sca~ of the abdomen - axial showing a left para-aortic tumour isoaense to skeletal muscle. margins were lobulated and clear. There was a marked homogeneous enhancement post contrast except for a small hypodense area at the inferior pole thought to be cystic necrosis. There was no infiltration or compres- sion of surrounding structures (Fig. 3.) A second tumour was found in the left adrenal gland measured 3.8 x 2.3 x Fig. 3. Reconstructed CT image - corona! show- ing left para-aortic mass just above the aortic bifur- cation - organ of Zuckerkandl. CASE REPORT Fig. 4. Contrast-enhanced CT - axial showing markedly enhancing extra-adrenal phaeochromo- cytoma, left para-aortic region. Fig. 5. Contrast-enhanced CT of the abdomen. Coronal reconstruction shows left suprarenal tumour (adrenal phaeochromocytoma). 36.6 em. It was smooth and enhanced homogeneously and markedly post contrast (Fig. 4). The right adrenal gland was nor- mal. Both renal arteries were normal. No lymph node enlargement was noted. There were no liver or bone metastases. Coronal and sagittal mul- tiplanar reconstruction (MPR) aided in localisation and visualisation of tumours (Fig.5). Biopsy An excision biopsy was performed and two phaeochromocytomas removed. No postoperative complica- tions were reported. . Histology confirmed the diagnosis of phaeochromocytoma. Discussion Phaeochromocytoma is a subtype of the paragangliomas, a neuro- endocrine tumour that arises from paraganglionic tissue. I It is responsible for 0.1% of hyper- tension cases. Location It occurs anywhere in the sympa- thetic nervous system from the neck to the sacrum: (l; adrenal medulla (85 - 90%); (iz) extra-adrenal (10 - 15%), with the majority adjacent to the kid- neys; and (iii) other sites including the organ of Zuckerkandl, the gonads, bladder and thoracic para-vertebral region. Our patient's tumour was in the left adrenal gland and the organ of Zuckerkandl (extra-adrenal site adja- cent to aortic bifurcation). Clinical findings Symptoms are secondary to excess catecholamine production. Classical presentation is: (1; parox- ysmal (50%) or sustained (50%) hypertension accompanied by head- aches, tachycardia, diaphoresis, palpi- tations and anxiety, with 9% of patients asymptomatic;' (ii) elevated vanillylmandelic acid in 24-hour urine and metanephrines (these were normal in our patient); and (iit) ele- vated serum catecholamines. 1-4 RuleoflO This postulates that 10% of cases are;'? (i) familial; (ii) children; (iil; bilateral adrenal; (iv) extra-adrenal ; (v) multiple; and (vi) malignant. Associations 1. Multiple endocrine neoplasia (phaeochromocytoma, usually bilat- eral and almost always intra-adrenal).' 23 SA JOURNAL OF RADIOLOGY • September 2002 • Sipple syndrome: Phaeochromo- cytoma, medullary carcinoma of the thyroid (MCT) and parathy- roid adenoma (multiple endocrine neoplasia (MEN) type II). • Mucosal neuroma syndrome (MEN type III) - MCT plus intestinal ganglioneuromatosis plus phaeochromocytoma. 2. Neuroectodermal disorders: Tuberous sclerosis, neurofibromato- sis,Von Hippel Lindau syndrome. 2 3. Familial phaeochromocytosis. 4. Carney's syndrome: Paragang- lioma plus gastric epitheloid leiomyo- sarcoma plus pulmonary chon- dioma.' Imaging Plain X-rays or IVU with tomo- grams may reveal a mass above the kidney with renal involvement but in our case it did not help. Both are low in sensitivity and specificity,' Ultrasound is helpful in children where it has proved to be highly sue- cessful.' A well-marginated solidI complex cystic tumour, homo or het- erogeneous is found in the adrenal or • 1·3para-aortic areas. CT scan. Localisation is accurate in 91% of cases with tumours> 2 ern.' Sensitivity is 93 - 100%. It is the method of choice. Detection rates are high. The small number of failures tend to occur in children with poorly developed fat planes and in cases of extra-adrenal phaeochromocytoma. CT shows adrenal/extra-adrenal mass with strong contrast enhancement. Low density areas are due to necrosis or haemorrhage.' Calcifications are rare. NB: N contrast injection may pre- cipitate hypertensive crisis in patients not on alpha-adrenergic blockers.' In CASE REPORT our case the patient was already on treatment. MR. The detection rate is also high with MRI and it may eventually replace CT as the investigation of choice since it involves no radiation.' Its use is limit- ed by cost and availability. 2 Phaechromocytomas are extremely hyperintense on T2Wl and show marked enhancement post contrast. Intensity is considerably higher when compared with adenoma and metas- tases.' Nuclear medi- cine studies Scintigraphy with MIBG is 80 - 90% sensitive, and 98% specific,' It is useful in detection of extra- adrenal tumours or when adrenals are normal on CT and MRI. False-posi- tive and false-negative results have both been recorded.' In our patient MIBG showed no evidence of phaeochromocytoma; false-negative result. Arteriography and venous sampling These are occasionally used in the search for ectopic tumours.' Arteriography: Localisation is useful in more than 90%. Usually hypervascular lesion with intense tumour blush enlarged feeding arter- ies and neovascularisation.' Complications Malignancy in 10% of lymph nodes, liver, lungs, and bone. Metastases may be hormonally active.' References 1. Weissleder R, Rieumont MJ, Wittenberg J. Primer of Diagnostic Imaging. 2nd ed. St Louis: Mosby,1997: 293 - 294. 2. Dahnert W Radiology Review Manual. 4th ed. Baltimore: Lippincott,1999: 781-782. 3. Sutton D. Textbook of Radiology and Imaging. 6th ed. vol 2. London: Churchill Livingstone, 1997: 1077-1082. 4. Grainger RG, Allison D. Diagnostic Radiology. 2nd ed. London: Churchhill Livingstone, 1997: 1170-1171. r NHS ~~ Lanarkshire Locum Consultant Radiologists (These posts may lead to permanent positions) With clinical services expanding as a result of the installation of a new 1.5T MRI scanner, Lanarkshire Acute Hospitals NHS Trust has opportunities for Consultant Radiologists to work at either our brand new Wishaw General Hospital or our recently rejuvenated hospital at Monklands. A new PACS system covering all sites within the Trust combined with our Doppler ultrasound scanners, a multislice CT scanner and mammography will all bring Lanarkshire to the leading edge of imaging technology and patient management. At Lanarkshire Acute Trust, we know that not everyone can commit themselves to a 'normal' working week. There are those who can make a valuable contribution, but only on a part-time or job- share basis. That's why we go out of our way to provide the flexibility that's increasingly demanded in the modern workplace, so you can take advantage of our brand new hospitals and radiology facilities, and at the same time develop your career at your own time and pace. Preferably holding or working towards the FRCR or equivalent, you will also have completed higher specialist training in Radiology as well as possess a CCST in Radiology or equivalent. Centrally located for Glasgow and Edinburgh, Lanarkshire boasts affordable housing and excellent educational facilities. Informal enquiries will be welcomed by Dr T J Nunn, Associate Medical Director on 01698 366549 or Dr J Roberts, Clinical Director for Radiology for Wishaw General Hospital on 01698 366514 and Dr A J Naismith, Associate Medical Director on 01236 712308 or Dr J Guse, Clinical Director, Imaging Services on 01236 712175 for Monklands Hospital. An information pack is available from the Senior Personnel Manager, MedicalPersonnel Department, Wishaw General Hospital, 50 Netherton Street, Wishaw ML2 OOP. Telephone +44 (0) 1698 366551. Fax +44 (0) 1698 366554. Please quote ref MW276. Closing date 27 September 2002. 24 SAJOURNAL OF RADIOLOGY. September2002