Pheochromocytoma with renal artery stenosis: A case-based review of literature

Kota, Sunil Kumar; Kota, Siva Krishna; Meher, Lalit Kumar; Tripathy, Prabhas Ranjan; Jammula, Sruti; Modi, Kirtikumar D

doi:10.4103/0975-3583.91601

Cited by 6 publications

(5 citation statements)

References 14 publications

(15 reference statements)

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“…The pathophysiologic mechanisms involved in this combination include the compression of the renal artery by ipsilateral tumor, persistent arterial vasospasm due to catecholamines excess, atherosclerosis secondary to high blood pressure and disturbed lipid profile resulting from catecholamines excess, generalized neuroectodermal dysplasia and finally a periarterial adhesion complicating tumoral resection. In some cases, this association may be a chance phenomenon where renal artery stenosis is secondary to an independent etiology like fibromuscular dysplasia [ 10 ]. Importance of preoperative diagnosis of the pheochromocytoma and renal artery stenosis is clear.…”

Section: Discussionmentioning

confidence: 99%

Rare association of aortoarteritis and pheochromocytoma: A case report

Toutai

Berrajaa

Aissaoui

et al. 2020

International Journal of Surgery Case Reports

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Highlights Pheochromocytoma is a great masquerador. The coexistence of pheochromocytoma along with both aortoarteritis and renal artery stenosis is very rare. Management of vascular abnormalities is based on the control of catecholamine release. Pheochromocytoma should be included as differential diagnosis of aortoarteritis.

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Section: Discussionmentioning

confidence: 99%

Rare association of aortoarteritis and pheochromocytoma: A case report

Toutai

Berrajaa

Aissaoui

et al. 2020

International Journal of Surgery Case Reports

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“…We describe the unusual association between two very rare conditions causing juvenile hypertension, bilateral PHEO and reninoma, in a patient affected by MEN2B and ACE-polymorphism. The coexistence of PHEO and hyperreninemia secondary to renal artery stenosis has been previously reported [ 6 ], while only a paper, which described a 18 years follow-up in a family affected by MEN 2 B, found the association between adrenal medullary hyperplasia and renin-secreting juxtaglomerular tumor [ 7 ]. Furthermore, we discovered in this patient the presence of ACE gene I/D polymorphism which is known to be associated with cardiovascular risk.…”

Section: Discussionmentioning

confidence: 99%

A rare case of juvenile hypertension: coexistence of type 2 multiple endocrine neoplasia -related bilateral pheochromocytoma and reninoma in a young patient with ACE gene polymorphism

et al. 2015

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BackgroundPheochromocytoma and reninoma represent two rare diseases causing hypertension. We here reported a rare case of association between type 2 multiple endocrine neoplasia related bilateral pheochromocytoma and reninoma. Moreover, polymorphism of ACE gene, which is known to be related to an increase of cardiovascular risk, has been found in the same patient.Case presentationA 24 year old Caucasian man came to our attention for severe hypertension, resistant to anti-hypertensive polytherapy. At the age of twenty he had undergone total thyroidectomy with lymphadenectomy for medullary carcinoma. Genetic testing showed a RET mutation of codon 918 (exon 16) not documented in other family members. During the follow-up, a progressive increase of urinary metanephrines and catecholamines was recorded. Our evaluation confirmed the presence of severe hypertension (220/140 mmHg) and a severe increase of urinary catecholamines and metanephrines. Due to the presence of hypokalemia, other causes of hypertension were researched leading to the discovery of hyperreninemia (236 μUI/ml) with mild hyperaldosteronism, and a mild increase of the renal artery resistance at ultrasound. An abdominal MRI showed multiple adrenal masses and a right kidney nodular lesion of about 2 cm.The patient underwent bilateral adrenalectomy and right nephrectomy, and histology confirmed the presence of bilateral pheochromocytoma and right reninoma. The post-surgery laboratory evaluation showed a rapid reduction of the urinary metanephrines while plasma renin level remained low in spite of the bilateral adrenalectomy without any mineralocorticoid supplementation. To further investigate these unusual feature, we performed genetic testing for the ACE gene, which revealed the presence of ACE I/D polymorphism.ConclusionThis unique report describes the association between two rare causes of hypertension in the same patient. Furthermore, the absence of requirement of mineralocorticoid supplementation in spite of bilateral adrenalectomy, represent an uncommon and interest finding.

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“…[78910] Two aspects make our report unusual: (i) the coexistence of pheochromocytoma with AAA in the patient and (ii) although there are case reports citing the association between pheochromocytoma and AAA,[111213] to our sincere belief, this is the first such report citing this uncommon association from India.…”

Section: Discussionmentioning

confidence: 99%

“…[6] Treatment depends on the size of the lesion. Although previous reports of AAA in association with pheochromocytoma exist in the literature,[789] a similar coexistence has not been reported in Indian patients till date. We report the case of a patient who presented with mutual existence of these two conditions.…”

Section: Introductionmentioning

confidence: 88%

Coexistence of pheochromocytoma with abdominal aortic aneurysm: An untold association

Kota

Jammula

et al. 2013

Ann Med Health Sci Res

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Pheochromocytomas have been described in association with rare vascular abnormalities, most common of them being renal artery stenosis. A 45-year-old woman was admitted to our hospital with complaints of headache, sweating, anxiety, dizziness, nausea, vomiting and severe hypertension. For the last several days, she was having a dull aching abdominal pain with a palpable, pulsatile, expansile and non-tender mass in the epigastric region. Hypertension was confirmed biochemically to result from excess catecholamine production. Abdominal computed tomography revealed the presence of a right adrenal pheochromocytoma. Magnetic resonance imaging of the abdomen demonstrated an abdominal aortic aneurysm (AAA) of maximum transverse diameter of 4.5 cm with 3 cm lumen. Surgical removal of pheochromocytoma resulted in normalization of blood pressure to normal. Because of the asymptomatic 4.5 cm aneurysm, our patient was advised for periodic follow-up. To our belief, this is the first such case report emanating from India, citing this rare association between pheochromocytoma and AAA. It is concluded that when the two diseases occur simultaneously, both must be diagnosed accurately and treated adequately. Possible mechanisms of such an uncommon association are also discussed.

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Pheochromocytoma with renal artery stenosis: A case-based review of literature

Cited by 6 publications

References 14 publications

Rare association of aortoarteritis and pheochromocytoma: A case report

Rare association of aortoarteritis and pheochromocytoma: A case report

A rare case of juvenile hypertension: coexistence of type 2 multiple endocrine neoplasia -related bilateral pheochromocytoma and reninoma in a young patient with ACE gene polymorphism

Coexistence of pheochromocytoma with abdominal aortic aneurysm: An untold association

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