J Korean Surg Soc.  2012 Jun;82(6):389-393.

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

• Yi JW
• Oh EM
• Lee KE
• Choi JY
• Koo DH
• Kim KJ
• Jung KC
• Kim SY
• Youn YK

Abstract

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 microg/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 microg/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.

Keyword

Pheochromocytoma; Paraganglioma; Adrenal glands; Dopamine; Adrenergic alpha-antagonists

MeSH Terms

Adrenal Glands
Adrenalectomy
Adrenergic alpha-Antagonists
Adult
Dopamine
Female
Humans
Korea
Paraganglioma
Pheochromocytoma
Porphyrins
Prognosis
Adrenergic alpha-Antagonists
Dopamine
Porphyrins

Figure

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  Fig. 1 Computed tomography scan shows hypervascular mass with dimensions of 4.3 × 3.2 cm on right adrenal gland with early washout enhancing pattern. Mass abuts against inferior vena cava (arrow).

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  Fig. 2 Magnetic resonance imaging showing 2.8 cm sized mass in right periadrenal area with slightly high signal intensity in T2 weighted image. Mass abuts against and compresses right adrenal gland laterally and inferior vena cava medially (arrow).

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  Fig. 3 Intraoperative view under videoscope. After careful dissection around tumor, tumor was removed successfully with assistance of surgeon's left hand (arrow).

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  Fig. 4 Microscopic findings (×40). (A) Hematoxylin/eosin staining revealed peritumoral adipose tissue extension and vascular invasion. Immunohistochemistry results were positive for neuroendocrine markers CD56 (B), synaptophysin (C), and chromogranin (D). The Ki-67 (E) index was less than 1%. S-100 (F) was negative.

Reference

1. Feldman JM, Blalock JA, Zern RT, Shelburne JD, Gaede JT, Farrell RE, et al. Deficiency of dopamine-beta-hydroxylase. A new mechanism for normotensive pheochromocytomas. Am J Clin Pathol. 1979. 72:175–185.

2. Proye C, Fossati P, Fontaine P, Lefebvre J, Decoulx M, Wemeau JL, et al. Dopamine-secreting pheochromocytoma: an unrecognized entity? Classification of pheochromocytomas according to their type of secretion. Surgery. 1986. 100:1154–1162.

3. Eisenhofer G, Goldstein DS, Sullivan P, Csako G, Brouwers FM, Lai EW, et al. Biochemical and clinical manifestations of dopamine-producing paragangliomas: utility of plasma methoxytyramine. J Clin Endocrinol Metab. 2005. 90:2068–2075.

4. Dubois LA, Gray DK. Dopamine-secreting pheochromocytomas: in search of a syndrome. World J Surg. 2005. 29:909–913.

5. Tam V, Ng KF, Fung LM, Wong YY, Chan MH, Lam CW, et al. The importance of the interpretation of urine catecholamines is essential for the diagnosis and management of patient with dopamine-secreting paraganglioma. Ann Clin Biochem. 2005. 42(Pt 1):73–77.

6. Awada SH, Grisham A, Woods SE. Large dopamine-secreting pheochromocytoma: case report. South Med J. 2003. 96:914–917.

7. Yasunari K, Kohno M, Yoshikawa J. A dopamine-secreting pheochromocytoma. Am J Med. 1999. 106:599–600.

8. Foo SH, Chan SP, Ananda V, Rajasingam V. Dopamine-secreting phaeochromocytomas and paragangliomas: clinical features and management. Singapore Med J. 2010. 51:e89–e93.

9. Sata N, Shiozawa M, Suzuki A, Kurihara K, Ohki J, Nagai H. Retroperitoneal hand-assisted laparoscopic surgery for endoscopic adrenalectomy. Surg Endosc. 2006. 20:830–833.

10. Schlumberger M, Gicquel C, Lumbroso J, Tenenbaum F, Comoy E, Bosq J, et al. Malignant pheochromocytoma: clinical, biological, histologic and therapeutic data in a series of 20 patients with distant metastases. J Endocrinol Invest. 1992. 15:631–642.