Adrenal pheochromocytoma with contralateral cortisol-producing adrenal adenoma: diagnostic and therapeutic management.
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.
Department of General and Visceral Surgery, University Hospital, Moorenstrasse 5, 40225 Düsseldorf, Germany. achim.wolf@med.uni-duesseldorf.de
A
A
Wolf
H S
HS
Willenberg
K
K
Cupisti
M
M
Schott
H
H
Geddert
A
A
Raffel
S R
SR
Bornstein
W A
WA
Scherbaum
W T
WT
Knoefel
D000306:Adrenal Cortex Neoplasms / Q000000981:diagnostic imaging* / Q000378:metabolism / Q000601:surgery; D000315:Adrenalectomy; D018246:Adrenocortical Adenoma / Q000000981:diagnostic imaging* / Q000378:metabolism / Q000601:surgery; D005260:Female; D006801:Humans; D006854:Hydrocortisone / Q000096:biosynthesis; D010535:Laparoscopy; D008875:Middle Aged; D010673:Pheochromocytoma / Q000000981:diagnostic imaging* / Q000378:metabolism / Q000601:surgery; D014057:Tomography, X-Ray Computed
D006854:Hydrocortisone