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DOI: 10.1055/s-2005-870159
Adrenal Pheochromocytoma with Contralateral Cortisol-producing Adrenal Adenoma: Diagnostic and Therapeutic Management
Publikationsverlauf
Received 1 February 2005
Accepted after revision 28 February 2005
Publikationsdatum:
07. Juli 2005 (online)
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Abstract
There is evidence for a close interrelation between the adrenomedullary and adrenocortical tissues, and there are well-characterized models of their paracrine interaction. To contribute to the studies of systemic interactions between these tissues, we studied a 52-year-old female patient with a pheochromocytoma and a contralateral cortisol-producing adenoma. Due to a misunderstanding, she presented to her family doctor to have an inherited kidney disease ruled out. An adrenal mass was discovered incidentally by ultrasound. A computerized tomography of the abdomen revealed bilateral adrenal masses. Due to excess catecholamine secretion, bilateral pheochromocytomas based on multiple endocrine neoplasia syndrome were suspected. Laboratory work-up, selective adrenal venous sampling and magnetic resonance imaging studies established the diagnosis of a pheochromocytoma in the right-hand adrenal gland and a cortisol-producing adenoma on the left. Simultaneous bilateral laparoscopic subtotal adrenalectomy was performed. Immunohistochemistry showed positive staining against chromogranin A in a histological specimen obtained from the right-hand adrenal gland, while the left was negative; the left-hand adrenal gland stained positive against the ACTH receptor (MC2R) while the right was negative. Genetically, the patient was negative for MEN2, von Hippel-Lindau disease, and mutations in subunits B, C, and D of the succinate dehydrogenase gene. Although presence of bilateral adrenal adenomas or bilateral adrenal pheochromocytomas in certain inherited disorders are possible, this rare case of an adrenal pheochromocytoma combined with a contralateral cortisol-producing adrenal adenoma may further underline the wide range of complex interactions between the two endocrine systems.
Key words
Adrenal tumor - Incidentaloma - Pheochromocytoma - Cortisol-producing adenoma - Hypercortisolism - Cushing’s syndrome
References
- 1 Koch C A, Pacak K, Chrousos G P. The molecular pathogenesis of hereditary and sporadic adrenocortical and adrenomedullary tumors. J Clin Endocrinol Metab. 2002; 87 (12) 5367-5384
- 2 Neumann H PH, Bausch B, McWhinney S R, Bender B U, Gimm O, Franke G, Schipper J, Klisch J, Altehoefer C, Zerres K, Januszewicz A, Eng C, Smith W M, Munk R, Manz T, Glaesker S, Apel T W, Treier M, Reineke M, Walz M K, Hoang-Vu C, Brauckhoff M, Klein-Franke A, Klose P, Schmidt H, Maier-Woelfle M, Peczkowska M, Szmigielski C, Eng C. Germ-line mutations in nonsyndromic pheochromocytoma. N Engl J Med. 2002; 346 (19) 1459-1466
- 3 Stratakis C A. Genetics of adrenocortical tumors: gatekeepers, landscapers and conductors in symphony. Trends Endocrinol Metab. 2003; 14 (9) 404-410
- 4 Stratakis C A, Bossis I. Genetics of the adrenal gland. Rev Endocr Metab Disord. 2004; 5 (1) 53-68
- 5 Gimm O, Koch C A, Januszewicz A, Opocher G, Neumann H P. The genetic basis of pheochromocytoma. Front Horm Res. 2004; 31 45-60
- 6 Marx S J, Stratakis C A. Multiple endocrine neoplasia - introduction. J Intern Med. 2005; 257 (1) 2-5
- 7 Bornstein S R, Ehrhart-Bornstein M, Scherbaum W A. Ultrastructural evidence for cortico-chromaffin hybrid cells in rat adrenals?. Endocrinology. 1991; 129 1113-1115
- 8 Vrezas I, Willenberg H S, Mansmann G, Hiroi N, Fritzen R, Bornstein S R. Ectopic adrenocorticotropin (ACTH) and corticotropin-releasing hormone (CRH) production in the adrenal gland: Basic and clinical aspects. Microsc Res Techniq. 2003; 61 (3) 308-314
- 9 Seppel T, Schlaghecke R. Augmented 17 alpha-hydroxyprogesterone response to ACTH stimulation as evidence of decreased 21-hydroxylase activity in patients with incidentally discovered adrenal tumours (‘incidentalomas’). Clin Endocrinol. 1994; 41 (4) 445-451
- 10 Willenberg H S, Mansmann G, Fritzen R, Schott M, Feldkamp J, Schulte K M, Scherbaum W A, Bornstein S R. The short synacthen test in the evaluation of adrenal masses in patients with malignancies. Endocr Res. 2002; 4 (28) 793-797
- 11 Willenberg H S, Stratakis C A, Marx C, Ehrhart-Bornstein M, Chrousos G P, Bornstein S R. Aberrant interleukin-1 receptors in a cortisol-secreting adrenal adenoma causing Cushing’s syndrome. N Engl J Med. 1998; 339 27-31
- 12 Yang C Y, Chou C W, Lin M B, Lin C N, Wang L W, Chen S Y, Cheng H M. Recurrent pulmonary embolism in an elderly patient with Cushing’s syndrome, adrenocortical adenoma, pheochromocytoma and prostate adenocarcinoma. J Chin Med Assoc. 2004 Jul; 67 (7) 360-365
- 13 Morimoto S, Sasaki S, Moriguchi J, Miki S, Kawa T, Nakamura K, Itoh H, Nakata T, Takeda K, Nakagawa M. Unique association of pheochromocytoma with contralateral nonfunctioning adrenal cortical adenoma. Am J Hypertens. 1998; 11 117-121
- 14 Yotsuyanagi S, Fuse H, Koshida K, Uchibayashi T, Namiki M. A case of adrenal pheochromocytoma with contralateral adrenocortical adenoma. Hinyokika Kiyo. 2001; 47 89-93
- 15 Ehrhart-Bornstein M, Hinson J P, Bornstein S R, Scherbaum W A, Vinson G P. Intraadrenal interactions in the regulation of adrenocortical steroidogenesis. Endocr Rev. 1998; 19 101-143
- 16 Bornstein S R, Breidert M, Ehrhart-Bornstein M, Kloos B, Scherbaum W A. Plasma catecholamines in patients with Addison’s disease. Clin Endocrinol. 1995; 42 215-218
- 17 Merke D P, Chrousos G P, Eisenhofer G, Weise M, Keil M F, Rogol A D, Van Wyk J J, Bornstein S R. Adrenomedullary dysplasia and hypofunction in patients with classic 21-hydroxylase deficiency. N Engl J Med. 2000; 343 1362-1368
- 18 Janetschek G, Finkenstedt G, Gasser R, Waibel U G, Peschel R, Bartsch G, Neumann H PH. Laparoscopic surgery for pheochromocytoma: Adrenalectomy, partial resection, excision of paragangliomas. J Urol. 1998; 160 (2) 330-334
- 19 Inabnet W B, Pitre J, Bernard D, Chapuis Y. Comparison of the hemodynamic parameters of open and laparoscpoic adrenalectomy for pheochromocytoma. World J Surg. 2000; 24 574-578
- 20 Wolf A, Goretzki P E, Röhrborn A, Feldkamp J, Simon D, Scherbaum W A, Röher H D. Phaeochromocytoma during pregnancy: Laparoscopic and conventional surgical treatment of two cases. Exp Clin Endocrinol Diabetes. 2004; 112 98-101
- 21 Walz M K, Peitgen K, Walz M V, Hoermann R, Saller B, Giebler R M, Jockenhovel F. Posterior retroperitoneoscopic adrenalectomy: lessons learned within five years. World J Surg. 2001; 25 728-734
- 22 Grumbach M M, Biller B MK, Braunstein G D, Campbell K K, Carney J A, Godley P A. et al . NIH CONFERENCES: Management of the Clinically Inapparent Adrenal Mass (“Incidentaloma”). Annals of Internal Medicine. 2003; 138 424-430
- 23 Mansmann G, Lau J, Balk E, Rothberg M, Miyachi Y, Bornstein S R. The Clinically Inapparent Adrenal Mass: Update in Diagnosis and Management. Endocr Rev. 2004; 25 (2) 309-340
A. Wolf, M. D.
Department of General and Visceral Surgery, University Hospital of Düsseldorf
Moorenstraße 5 · 40225 Düsseldorf · Germany
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eMail: achim.wolf@med.uni-duesseldorf.de