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Year : 2001  |  Volume : 17  |  Issue : 2  |  Page : 161-163

Extra-adrenal pheochromocytoma - a case report

Department of Urology, Sir J J Hospital, Mumbai, India

Correspondence Address:
Percy Jal Chibber
Department of Urology, Sir J J Hospital, Byculla, Mumbai - 400 008
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Source of Support: None, Conflict of Interest: None

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Keywords: Pheochromocytoma; Extra-Adrenal.

How to cite this article:
Somani SH, Bhawani JB, Chibber PJ. Extra-adrenal pheochromocytoma - a case report. Indian J Urol 2001;17:161-3

How to cite this URL:
Somani SH, Bhawani JB, Chibber PJ. Extra-adrenal pheochromocytoma - a case report. Indian J Urol [serial online] 2001 [cited 2022 Jun 30];17:161-3. Available from:

   Case Report Top

A 28-year-old female presented with headache and rest­lessness for 2 years and vague abdominal pain for 6 months. Investigations revealed a mass below the lower pole of the right kidney on ultrasonography. An attempt at removal of the mass was attended by severe hypertensive crisis man­dating abandonment of the procedure. Patient was referred to our center for further management. On admission, her blood pressure was 220/120 mm of Hg. Urinary Vanillylmandelic acid (VMA) was 15.5 mg (normal range 2-14 mg). Computerised tomography (CT) abdomen re­vealed a 5-cm diameter mass below the lower edge of the right kidney near the midline with solid and cystic compo­nent and enhancement on post-contrast study. Adrenals were normal. 131 I-Metaiodobenzylguinidine (MIBG) scan re­vealed a single area of increased uptake at the site of the mass [Figure - 1]. With adequate preoperative preparation ex­ploratory laparotomy was done. A mass below the lower pole of right kidney, in front of the inferior vena cava and aorta below the origin of inferior mesenteric artery was found and removed [Figure - 2]. Histology of the mass was con­sistent with pheochromocytoma [Figure - 3]. Patient was asymp­tomatic postoperatively and her blood pressure was well controlled on 25 mg atenolol orally daily. Follow-up uri­nary VMA was normal and MIBG scan after 3 months re­vealed no area of increased tracer uptake.

   Discussion Top

Pheochromoeytoma is rare, accounting for less than 0.1 % of hypertensive population. [2] Extra-adrenal pheochro­mocytomas are rarer still (10% of all pheochromocyto­mas) and are usually seen in the second and third decades. [1] There is no sex predominance. [1] Extra-adrenal pheochro­mocytomas are often multicentric and more likely to be malignant than those of adrenal origin. [1]

The organ of Zuckerkandl, small collections of paragan­glion tissue that lie contiguous to the aorta between the root of inferior mesenteric artery and the aortic bifurcation, were described by Emil Zuckerkandi in 1901. [1],[3] The embryo­logical origin and histology of these tissues are similar to the adrenal medulla and the tumours arising from both of them are functionally similar. [3] Organ of Zuckerkandl is sec­ond in frequency as a site for extra-adrenal pheochromocy­toma. [1] These tumours are predominantly epinephrine producing as opposed to other extra-adrenal sites which are norepinephrine-producing tumours. [1],[2]

The presence of hypertension with symptoms of adren­ergic excess should alert the clinician to the tumours of adrenal medulla as well as the extra-adrenal paraganglion system. [1] Patients with extra-adrenal pheochromocytoma commonly present with the classic triad of headache, pal­pitations and sweating. Sustained or paroxysmal hyper­tension is present in 80-100% of these patients.

Urinary estimation of catecholamines and their metabolites is a better biochemical investigation than plasma estimation for the diagnosis of these lesions. [1] CT scan is the imaging mo­dality of choice for localization. [1],[2] MIBG scan is indicated in extra-adrenal tumours to rule out multicentricity and metasta­sis. [1],[2] In extra-adrenal pheochromocytomas the traditional his­tological markers of malignancy may not predict the malignant behavior. The diagnosis of malignancy can be made with con­fidence only by demonstrating tumour cells at sites where para­ganglion tissue is normally absent. [1],[2] Surgery after adequate preoperative preparation remains the treatment of choice. [1]

A lifelong follow-up is indicated, as the extra-adrenal pheochromocytomas are more likely to recur and to meta­stasize. Annual determination of urinary catecholamines and their metabolites is recommended. Persistent hyper­tension after successful removal of pheochromocytoma occurs in approximately 25% of cases. [1]

   References Top

1.Whalen RK, Althausen AF, Daniels GH. Extra-adrenal pheochro­mocytoma. J Urol 1992: 147: 1-10.  Back to cited text no. 1    
2.Goldfarb DA, Novick AC. Bravo L et al. Experience with extra­ adrenal pheochromocytorna. J Urol 1989; 142: 931-936.  Back to cited text no. 2    
3.Brantigen CO, Katase RY. Clinical and pathologic features of paragangliomas of the organ of Zuckerkandl. Surgery 1969; 65: 898.  Back to cited text no. 3    


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


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