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.
Wolf
A
A
Department of General and Visceral Surgery, University Hospital, Moorenstrasse 5, 40225 Düsseldorf, Germany. achim.wolf@med.uni-duesseldorf.de
Willenberg
H S
HS
Cupisti
K
K
Schott
M
M
Geddert
H
H
Raffel
A
A
Bornstein
S R
SR
Scherbaum
W A
WA
Knoefel
W T
WT
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