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Year : 2018  |  Volume : 5  |  Issue : 3  |  Page : 239-241

Renal hilar pheochromocytoma with nonfunctional kidney

Department of Urology, Christian Medical College, Ludhiana, Punjab, India

Date of Web Publication17-Jul-2018

Correspondence Address:
Kim J Mammen
Department of Urology, Christian Medical College, Ludhiana - 141 008, Punjab
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/cjhr.cjhr_123_17

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Pheochromocytomas are neuroendocrine tumors derived from embryonic neural crest cells. They are mostly found in the adrenal glands (pheochromocytomas) and sometimes in the extra-adrenal paraganglia (paragangliomas) of the autonomic nervous system. Paragangliomas arising from the kidney or in the renal hilus are very rare, and preoperative diagnosis is rarely made.We present the case of a 38-year-old hypertensive female, presenting with dull aching, nonradiating right flank pain for 2 months and no other symptoms. Computed tomography demonstrated a well-defined, hypodense enhanced lesion located on the hilum of the right kidney. Laparotomy was performed, and histology of the excised mass revealed an extra-adrenal pheochromocytoma. The intervention was effective, and the patient is doing fine.

Keywords: Immunohistochemistry, nephrosclerosis, paraganglioma, pheochromocytoma

How to cite this article:
Tuli A, Kaur N, Mammen KJ. Renal hilar pheochromocytoma with nonfunctional kidney. CHRISMED J Health Res 2018;5:239-41

How to cite this URL:
Tuli A, Kaur N, Mammen KJ. Renal hilar pheochromocytoma with nonfunctional kidney. CHRISMED J Health Res [serial online] 2018 [cited 2020 Feb 24];5:239-41. Available from: http://www.cjhr.org/text.asp?2018/5/3/239/236887

  Introduction Top

Extra-adrenal pheochromocytoma (EAP) commonly occurs below the diaphragm, in the organ of Zuckerkandl and sometimes in the retroperitoneum. EAP, arising along the sympathetic, and parasympathetic paraganglia, are called paraganglioma.[3] They comprise about 15% of chromaffin cell tumors and only 0.1% cases are associated with hypertension. EAP comprise about 15% of adult and 30% of childhood pheochromocytomas.[2]

Usually they are found in the abdomen, pelvis, thorax, neck, and skull base. We report a rare case of EAP from the renal hilum compressing the renal artery causing hypertension and a nonfunctional kidney.

  Case Report Top

A 38-year-old female with hypertension was admitted with dull aching, nonradiating right flank pain for 2 months. She was on Amlodipine 5 mg twice daily. She was evaluated elsewhere for similar complaints 3 years earlier, and an ultrasound revealed a 3 cm × 3 cm lesion in the right renal hilum. She was treated nonoperatively, but on subsequent follow-up, the hilar mass remained unchanged. On physical examination, she had tenderness in the right flank. Vital signs included a normal pulse rate with blood pressure (BP) of 140/100 mmHg. Ultrasound abdomen revealed a small right kidney with hilar space occupying lesion. On contrast-enhanced Computed tomography (CT) scan, a well-defined hypodense, enhancing lesion 3.0 cm × 2.9 cm was seen in the right renal hilum partially occluding the renal artery [Figure 1] and [Figure 2], which was suggestive of a renal hilar thrombosed aneurysm with nonfunctioning of ipsilateral kidney. DTPA renogram showed a glomerular filtration rate of 8.6 ml/min in the right kidney. Preoperatively, hypertension was controlled with amlodipine 5 mg and metoprolol 25 mg. The patient was taken up for a right nephrectomy with the excision of the mass. Intraoperatively, she had 3 cm × 3 cm hilar mass adherent to inferior vena cava (IVC) and the adjoining renal capsule and parenchyma. Intraoperatively, while dissecting the hilar mass, the patient had tachycardia and high BP recordings which were controlled with incremental doses of labetalol. The mass was resected along with right nephrectomy. During the procedure, a small iatrogenic rent in the IVC was repaired with 5-0 prolene continuous hemostatic sutures. Her postoperative course was uneventful. Histopathologic features [Figure 3] and [Figure 4] revealed pheochromocytoma with a small right contracted kidney with benign nephrosclerosis and chronic pyelonephritis. On follow-up, she is doing well and is normotensive without any antihypertensive drugs.
Figure 1: A well-defined hypodense, enhancing lesion 3.0 cm × 2.9 cm in the right renal hilum, no calcification or necrosis seen, displacing renal artery, ureter posteriorly, and renal vein anteriorly. Inferior vena cava noted in close relation to the mass

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Figure 2: A well-defined enhancing lesion 3.0 cm × 2.9 cm in the renal hilar region with mass effect on the renal vasculature causing shrunken kidney on the right side

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Figure 3: Low power microphotograph showing tumor comprised of polygonal cells arranged in small nests and sheets H and E, ×100

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Figure 4: Characteristic Zellballen pattern with small nests of tumor cells having round to oval nuclei, prominent nucleoli (arrow) H and E, ×400

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  Discussion Top

Pheochromocytomas comprise 0.3%–0.95% of neuroendocrine tumors which arise from chromaffin sympathetic nervous system. They release catecholamines in excess amounts and cause hypertension.[1] Before surgical removal of the tumors, the optimization of BP, as well as intravascular volume, is an important measure to avoid and suppress perioperative adverse hemodynamic events.[4]

Since our patient presented with right flank pain for 2 months with a well-controlled BP on a single drug for 10 years and considering normal adrenals in CT scan, the space-occupying lesion in the renal hilum was not suspected to be a pheochromocytoma. The well-defined margin of the lesion and its stable size was suggestive of benign renal hilar space occupying lesion, but due to its close approximation to the major vessels, a thrombosed aneurysm was also suspected. As there was no obvious evidence of catecholamine overproduction, blood and urine catecholamines were not measured before the surgery.

Symptoms of pheochromocytoma occurred intraoperatively when there was an alteration in pulse and BP of the patient at the time of dissection of mass, and the histopathological examination proved the diagnosis. Extra-adrenal chromaffin tumors are called paragangliomas. Their incidence is 0.40–2.06 per million per year and most patients are between 30 and 40 years.[3] The clinical features of these paragangliomas are nonspecific and thus to diagnose, we need a high index of suspicion, especially in young hypertensives with space-occupying lesions around the sympathetic chain. Histology and immunohistochemistry remain the gold standard for making the definitive diagnosis.[3]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Garancini M, Cesana G, Bella CD, Tancredi A, Valtorta A, Giovenzana ME, Beltramo M, et al. Non-functioning paraganglioma located at the renal hilus: Case report. Biol Syst Open Access 2012;1:101.  Back to cited text no. 1
Pacak K, Eisenhofer G, Ahlman H, Bornstein SR, Gimenez-Roqueplo AP, Grossman AB, et al. Pheochromocytoma: Recommendations for clinical practice from the first international symposium. October 2005. Nat Clin Pract Endocrinol Metab 2007;3:92-102.  Back to cited text no. 2
Bode R, Agolli M, Domi R, Alimehmeti M. Renal hilar paraganglioma – A case report. Int J Sci 2015;4:120-2.  Back to cited text no. 3
Ramachandran R, Rewari V. Current perioperative management of pheochromocytomas. Indian J Urol 2017;33:19-25.  Back to cited text no. 4
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