PEDIATRIC SURGERY UPDATE ©

VOL 38 NO 01 JANUARY 2012

Dopamine-producing Pheochromocytoma

Pheochromocytomas are neuroendocrine tumors that produce, metabolize and secrete catecholamines such as norepinephrine and epinephrine. In very rare occasions hormonally active pheochromocytomas can produce and secrete only dopamine as the only catecholamine. Predominance of dopamine in this tumors and lack of production of other catecholamines is due to deficiency in tumor cells of dopamine-B-hydroxylase, the enzyme that converts dopamine into norepinephrine. Dopamine-producing pheochromocytomas are usually found as extraadrenal paragangliomas. Clinically patients harboring these tumors are normotensive. Most patients are diagnosed after identifying the adrenal or extraadrenal position of the tumor during incidental imaging or as the result of symptoms caused by the space-occupying tumor. Due to lack of hormonally associated symptoms these tumors can grow into large size. Tumors that produce dopamine can be identified by high plasma or urine concentrations of dopamine, or high plasma levels of free methoxytyramine. The incidence of malignancy is higher in this type of pheochromocytoma. Nausea, vomiting, flushing and orthostatic hypotension are a few of the symptoms exhibited by these patients. MRI is the imaging of choice to localize these tumors. Management consists of resection of the tumor. Persistent elevation of blood pressure after tumor resection have been documented.  PET-Scan is more specific than MIBG  in localizing residual disease.

References:
1- Minamiguchi N, Inui E, Nukui M: [A case of dopamine-secreting pheochromocytoma]. Hinyokika Kiyo. 45(12):831-3, 1999
2- Yasunari K, Kohno M, Minami M, Kano H, Ohhira M, Nakamura K, Yoshikawa J: A dopamine-secreting pheochromocytoma. J Cardiovasc Pharmacol. 36 Suppl 2:S75-7, 2000
3- Awada SH, Grisham A, Woods SE: Large dopamine-secreting pheochromocytoma: case report. South Med J. 96(9):914-7, 2003
4- Eisenhofer G, Goldstein DS, Sullivan P, Csako G, Brouwers FM, Lai EW, Adams KT, Pacak K: Biochemical and clinical manifestations of dopamine-producing paragangliomas: utility of plasma methoxytyramine. J Clin Endocrinol Metab. 90(4):2068-75, 2005
5- Tam V, Ng KF, Fung LM, Wong YY, Chan MH, Lam CW, Tam S, Lam CW: The importance of the interpretation of urine catecholamines is essential for the diagnosis and management of patient with dopamine-secreting paraganglioma. Ann Clin Biochem. 42(Pt 1):73-7, 2005
6- Foo SH, Chan SP, Ananda V, Rajasingam V: Dopamine-secreting phaeochromocytomas and paragangliomas: clinical features and management. Singapore Med J. 51(5):e89-93, 2010
7- Dubois LA, Gray DK: Dopamine-secreting pheochromocytomas: in search of a syndrome. World J Surg. 29(7):909-13, 2005

*Edited by: Humberto Lugo-Vicente, MD, FACS, FAAP
P.O. Box 10426, Caparra Heights Station, San Juan, Puerto Rico 00922-0426
Tel (787)-786-3495 Fax (787)-720-6103 E-mail: TITOLUGO@COQUI.NET
Internet Address:HTTP://HOME.COQUI.NET/TITOLUGO
® Copyright Office of the Library of Congress 1997


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Pediatric Surgery Update ISSN 1089-7739
Last updated: February 2012