Deficiency of Dopamine-<i>β</i>-hydroxlase: A New Mechanism for Normotensive Pheochromocytomas

Feldman, Jerome M.; Blalock, J; Zern, Ruthann T.; Shelburne, John D.; Gaede, Janet T.; Farrell, Ruth E.; Wells, Samuel A.

doi:10.1093/ajcp/72.2.175

Cited by 34 publications

(25 citation statements)

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“…Also Feldman et al [5] reported a normotensive patient with bilateral pheochromocytomas and elevated urinary excretion of homovanillic acid, the principal metabolite of dopamine. The authors speculated that clinical manifestations of the pheochromocytom a may result from the de ciency of tumour dopamine beta-hydroxylase activity leading to augmented dopamine secretion and decreased norepinephrine secretion.…”

Section: Discussionmentioning

confidence: 98%

Dopamine and Dopa Urinary Excretion in Patients with Pheochromocytoma–Diagnostic Implications

et al. 2001

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Pheochromocytoma, a potentially life-threatening disease, is a rare cause of hypertension. Most pheochromocytomas secrete excessive amounts of noradrenaline and adrenaline. It has been suggested by some authors that high circulating levels of dopamine and the catecholamine precursor dihydroxyphenylalanine (dopa) are more often associated with malignant rather than benign pheochromocytomas. Therefore the aim of this study was to evaluate urinary excretion of dopamine and dopa in patients with pheochromocytoma and to determine their role as a potential marker for malignancy of the tumour. We retrospectively analysed 120 consecutive patients (mean age 41 +/- 12 years) with histopathologically confirmed pheochromocytomas. All subjects were divided as follows: group 1 included patients with both elevated and normal dopamine urinary excretion; group 2 was characterized by increased and normal dopa urinary excretion. Dopamine urinary excretion was increased in all patients with malignant pheochromocytoma, but higher levels were also observed in some patients with a benign tumour included in group 1. Urinary excretion of dopa was in the normal range in all subjects with malignant pheochromocytoma. The results indicate that in some pheochromocytoma patients excessive dopamine excretion may point to malignant tumour, but is not a discriminating marker for malignancy in the whole studied group.

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Section: Discussionmentioning

confidence: 98%

Dopamine and Dopa Urinary Excretion in Patients with Pheochromocytoma–Diagnostic Implications

et al. 2001

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“…Extra-adrenal paragangliomas rarely secrete epinephrine, reflecting a decreased expression of phenylethanolamine-N-methyltransferase, the enzyme that converts norepinephrine to epinephrine (10). Tumors that A B produce dopamine either exclusively or predominantly are rare.…”

Section: Dopamine-secreting Paragangliomasmentioning

confidence: 99%

“…They are usually found as extra-adrenal paragangliomas (11) and can be located in the carotid body, retroperitoneum, and pelvis. The predominance of dopamine and the relative lack of production of other catecholamines in these tumors are due to the deficiency of dopamine-β-hydroxylase, the enzyme that converts dopamine to norepinephrine (10). In contrast to patients who harbor norepinephrine and/or epinephrine-secreting tumors, subjects with exclusively dopamine-producing tumors lack the classical presentation of paroxysms of headache, palpitations, diaphoresis, and hypertension.…”

Section: Dopamine-secreting Paragangliomasmentioning

confidence: 99%

Dopamine-secreting Carotid Body Paragangliomas-Biochemical Control with Radiotherapy

Soh

Kek

2012

Intern. Med.

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Head and neck paragangliomas that are exclusively or predominantly dopamine-secreting are rare. Surgery and/or radiotherapy are modalities for locoregional tumoral control. Little is known about the efficacy of radiotherapy for biochemical control in such tumors. We report a 62-year-old Chinese man with bilateral carotid body tumors which were exclusively dopamine secreting. The left-sided tumor invaded the skull base and encased the left carotid artery. Surgery was not performed due to high risk of morbidity and mortality. The patient received external beam radiotherapy to bilateral neck regions. Progressive decline and eventual normalization of urinary dopamine excretion was seen together with a slight reduction in tumor size. This is the first report demonstrating the efficacy of radiotherapy for both biochemical and locoregional control of functioning carotid body paragangliomas.

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“…It is usually found at extra-adrenal sites and is diagnosed as a subtype of paraganglioma. The high dopamine level in this tumor is caused by a deficiency in dopamine-beta-hydroxylase, which converts dopamine to norepinephrine [1]. For this reason, this tumor differs from classic pheochromocytomas in many respects, not only in its clinical features but also in its oncologic aspect.…”

Section: Introductionmentioning

confidence: 99%

An exclusively dopamine secreting paraganglioma in the retroperitoneum: a first clinical case in Korea

Lee

et al. 2012

J Korean Surg Soc

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Exclusively dopamine producing retroperitoneal paragangliomas are extremely rare. We have experienced the first Korean case managed successfully based on the proper evaluation. A 26-year-old female patient came to our attention after the accidental detection of an adrenal mass. She had no symptoms and denied any family history. Laboratory evaluations were normal but serum dopamine (425 ng/L) and 24-hour urine dopamine levels (1,565.3 µg/day) were elevated. She underwent laparoscopic right adrenalectomy. Histopathological diagnosis was a paraganglioma. After operation, dopamine levels in serum and 24-hour urine dropped to 0.09 ng/L and 388.4 µg/day. Dopamine producing paraganglioma elicit no clinical symptoms. Only the dopamine level is elevated in serum and 24-hour urine samples. Surgical resection without using preoperative alpha blockage is the treatment of choice. The prognosis for patients with this tumor tends to be poor because the diagnosis is usually delayed due to lack of symptoms.

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Deficiency of Dopamine-β-hydroxlase: A New Mechanism for Normotensive Pheochromocytomas

Cited by 34 publications

References 0 publications

Dopamine and Dopa Urinary Excretion in Patients with Pheochromocytoma–Diagnostic Implications

Dopamine and Dopa Urinary Excretion in Patients with Pheochromocytoma–Diagnostic Implications

Dopamine-secreting Carotid Body Paragangliomas-Biochemical Control with Radiotherapy

An exclusively dopamine secreting paraganglioma in the retroperitoneum: a first clinical case in Korea

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