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Year : 2004  |  Volume : 14  |  Issue : 3  |  Page : 291-293
Twin extra-adrenal pheochromocytoma

Department of Radiodiagnosis IG Medical College, Shimla, 171001 (H.P), India

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Keywords: Pheochromocytoma

How to cite this article:
Dhiman D S, Vyas S, Prakash S, Thakur J. Twin extra-adrenal pheochromocytoma. Indian J Radiol Imaging 2004;14:291-3

How to cite this URL:
Dhiman D S, Vyas S, Prakash S, Thakur J. Twin extra-adrenal pheochromocytoma. Indian J Radiol Imaging [serial online] 2004 [cited 2020 Aug 10];14:291-3. Available from:

   Introduction Top

Pheochromocytomas are catecholamine-secreting tumours, which arise from the chromaffin cells, affecting about 1 in 200,000 individuals in the general population. In 90% of cases pheochromocylomas arise in adrenal medulla Remaining 10% may arise from anywhere in the autonomic nervous system and are referred to as extra-ad­renal pheochromocytoma or paraganglioma. Pheochromocytomas follows 'rule of ten' i.e. approximately 10% are bilateral, multiple, extra adrenal, malignant, familial and occur in the children. Pheochromocytomas occur in both sexes equally, usually during early adult life or middle age. They are rare in chidren and accounts for about 2% of cases of secondary hypertension. The characteristic clinical symptoms include headache, palpitation, pallor, sweating, anxiety and tremors. Diagnosis is established by demonstration of increased catecholamine and their metabolies in urine blood. For localization of the tumor ultrasound, CT, MRI, Iodine - 123 MIBG scintigraphy are used.

   Case history Top

A l2years female child presented with a 5-month history of pain upper abdomen (epigastrium and left hypochondrium), which was mild to moderate in intensity, continuous and associated with projectile vomiting. There was also history of headache, cold hands and feet, facial flushing, sweating, palpitation and loss of weight. On clinical examination - patient was conscious, cooperative and well oriented.

No-pallor, icterus, cyanosis or lymphadenopathy

Pulse was 96/min. regular and good volume.

BP was persistently high ranging between 160/100 to 250/140 mm of Hg.

Haemogram - Hb-1 4gm%, TLC 8400 and ESR 35mm in 1 stsub hour.

Urine metanephrine were raised - 1.3mg/day. (Normal range is below 0.9mm/day). Urine Catecholamines were 0.9mg/day. (Normal range is 0.3-0.9mg/day).

   Radiological investigations Top

USG - Two homogenously solid, well defined, ovoid masses were seen on both side of abdominal aorta and adjacent to celiac axis. No necrosis or calcification was appreciated. Coeliac axis was stretched by bilateral masses and pancreas was pushed anteriorly. Liver, gallbladder, spleen, both kidneys and suprarenal regions were normal (Fiq. No.1).

IVP-Both kidneys functioned simultaneously. Both kidneys were normal in size, shape and outline. No displacement of kidneys was seen which is common finding in adrenal pheochromocytoma (Fig No.2)

CT - On unenhanced scans, two well defined, ovoid soft tissue density masses measuring 5 and 4.5 cm in diameter were seen on both side of abdominal aorta and adjacent to celiac axis (Fig. No.3). After intravenous contrast these masses revealed significant enhanement (100H.U.) with central necrosis. IVC was pushed posteriorly and to right Coeliac axis was stretched by bilateral masses and pancreas was pushed anteriorly. No infiltration into adjacent structure seen. Liver, gallbladder, spleen and both kidneys were normal (Fig. No.4).

On explorative laprotomy - Tumours adjacent to the celiac axis on both sides of abdominal aorta were identified which were extending to the paravertebral gutters. Tumours were markedly vascular. Right tumor was displacing the IVC posterolaterally and left one was lying over the aorta. Both adrenals were normal in size and location (Fig. No.5).

   Discussion Top

Adrenal pheochromocytomas are well defined, rounds or ovoid and homogenous or heterogeneous masses on sonography. Bowerman et al (1) in a series of eight surgically confirmed cases of pheochromocytomas found that most of these were either heterogeneous or homogenously solid. The heterogeneous lumours had areas of necrosis and hemorrhage. On unenhanced CT pheochromocylomas appear as round or oval; discrete masses of soft tissue density with central necrosis, calcification and cystic changes may be present in minority of cases (2). These tumours show intense enhancement after administration of intravenous contrast material. On MRI -T1 - weighted sequence are used to identify the low signal intensity mass and the high signal intensity seen on T2-weighted images helps to characterize it as pheochromocytomas (3) MIBG scanning can play a complementary role in evaluating adrenal pheochromocytoma. The combination of an adrenal mass seen on a CT or MRI and uptake on MIBG scanning is definitive in the diagnosis of pheochromocytoma (5).

Extra adrenal pheochromocytomas develop in the paraganglion chromaffin cells of the sympathetic nervous system and account for 10% of pheochromocytoma in adults and 30-40% in children. Extra adrenal pheochromocytomas occur at the para-adrenal area in over 37.5% of patients, in the organ of Zuckerkandel at the aortic bifurcation in 12.5%, in the chest 7.5%,5% each in inferior mesenteric artery, over left common iliac artery, in the bladder wall, and 2.5% each in celiac plexus, superior mesenteric artery, renal vessels, over right iliac artery, beneath the diaphragm and around the left ureter (6)

In 125% of patients, the exact location of the tumor could not be determined. Extra-adrenal tumours are usually sporadic. In adults these tumours are malignant in approximately 30-40% of patients compared to less than 2% in children. Extra-adrenal pheochromocytomas tend to be larger than their adrenal counterpart at the time of detection. Symptoms of extra-adrenal pheochromocytoma can be divided into two categories. The first category includes symptoms that are due to an excess of catecholamine and are similar to their adrenal counterpart. The second category includes symptoms that are due to the specific location of the tumours and may help in localizing the tumours. Since most of these tumours lie in the retroperitoneum it may be difficult to localize them by sonogrpahy because of body habitus and overlying gases. CT or MRI is more useful for -localization of these tumours[6].

   Conclusion Top

The present case is a unique in the sense that the extra-adrenal pheochromocytoma was twin, which is a rare entity, and present in both sympathetic chains and almost of same size that is not described in the literature as per our knowledge.

   References Top

1.Bowerman RA, Silver TM, Jaffe MH, et al. Sonogrpahy of adrenal pheochromocytomas. Radiology 1981;137:1227-1231.  Back to cited text no. 1  [PUBMED]  
2.Welch TJ, Sheedy PF, Heerden JAV, et al. Pheochromocytoma valueoo of computed tomography. Radiology 1983;148:501-503.  Back to cited text no. 2    
3.Quint LE, Glazer GM, Francis IR, et al. Pheochromocytoma and paraganglioma: Comparison of MR imaging with CT and 1-131 MIBG scintigrahy. Radiology 1987;165-89-93.  Back to cited text no. 3    
4.Dunnick NR. Adrenal imaging current status. AJR 1990;154:927-936.  Back to cited text no. 4  [PUBMED]  
5.Crillo RL, Bennet WF, Vitellas KM, et al. Pathology of the adrenal gland: Imaging features. AJR 1998;170:429-435.  Back to cited text no. 5    
6.Atiyeh BA, Barakat AJ, Abumrad NN, et al. Extra-adrenal pheochromocytoma. Journal of nephrology 1997;10(1):25-29.  Back to cited text no. 6    

Correspondence Address:
D S Dhiman
Valley View, Dhingu Road, Sanjauli, Shimla, 171 006(H.P)
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Source of Support: None, Conflict of Interest: None

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[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5]

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