Endocrinol Metab.  2012 Dec;27(4):323-328. 10.3803/EnM.2012.27.4.323.

A Case of Adrenal Cystic Pheochromocytoma with Contralateral Adrenocortical Adenoma Causing Subclinical Cushing's Syndrome

• Park CJ
• Seo JW
• Seoung HG
• Koh JH
• Lee YJ
• Kim BH
• Kim IJ

• KMID: 1973798
• DOI: http://doi.org/10.3803/EnM.2012.27.4.323

Abstract

Bilateral adrenal neoplasms are associated with metastatic cancer, pheochromocytoma and lymphoma. The coexistence of a unilateral functioning adrenocortical adenoma with contralateral pheochromocytoma is extremely rare. A 52-year-old woman complained of fatigue, headache, palpitation, and progressive weight gain. Hormonal assessment demonstrated high 24 hours urine epinephrine, norepinephrine, and free cortisol. A dexamethasone suppression test (overnight 1 mg, low dose 2 mg) showed insuppressible cortisol. Computerized tomographic scanning revealed a bilateral adrenal tumor. To preserve adrenal function, right adrenalectomy along with left adrenal tumorectomy was performed. Histological finding of the right adrenal tumor was pheochromocytoma and the left adrenal tumor was adrenocortical adenoma. This patient was the first case of a functional adrenocortical adenoma with contralateral cystic pheochromocytoma in Korea. We report the case with a review of the literature.

Keyword

Adrenocortical adenoma; Cushing syndrome; Pheochromocytoma

MeSH Terms

Adrenal Gland Neoplasms
Adrenalectomy
Adrenocortical Adenoma
Cushing Syndrome
Dexamethasone
Epinephrine
Fatigue
Female
Headache
Humans
Hydrocortisone
Korea
Lymphoma
Middle Aged
Norepinephrine
Pheochromocytoma
Weight Gain
Dexamethasone
Epinephrine
Hydrocortisone
Norepinephrine

Figure

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  Fig. 1 Preoperative computed tomography (CT) scan. (A) Abdomen CT reveals an 2.5 cm sized hypervascular adrenal mass in left adrenal gland, and (B) an 6.5 cm sized cystic mass with peripheral wall thickening in right adrenal gland.

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  Fig. 2 Preoperative 131I-metaiodobenzylguanidine (MIBG) scan. 131I-MIBG scan shows increased uptake in the right upper quadrant of the abdomen (corresponding the cystic mass in right adrenal area on computed tomography scan).

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  Fig. 3 (A, B) Histopathological finding of the right adrenal pheochromocytoma. Right tumor cells have round to oval nuclei and abundant granular amphophilic cytoplasm (H&E stain, × 400).

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  Fig. 4 Immunohistochemical staining of the right adrenal pheochromocytoma. The immunochemistry revealed positive reaction for neuron specific enolase (A, neuron specific enolase stain, × 400) and synaptophysin (B, synaptophysin stain, × 400).

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  Fig. 5 Histopathological finding of the left adrenocortical adenoma. Left adrenal adenoma was composed of zona reticularis like cells (A, H&E stain, × 40). They show compact cells with lipid sparse eosinophilic cytoplasm and clear cells with lipid sparse eosinophilic cytoplasm (B, H&E stain, × 100).

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