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Park, Seo, Seoung, Koh, Lee, Kim, and Kim: A Case of Adrenal Cystic Pheochromocytoma with Contralateral Adrenocortical Adenoma Causing Subclinical Cushing's Syndrome
Case Report
Endocrinology and Metabolism

Endocrinology and Metabolism 2012; 27(4): 323-328.

Published online: 20 December 2012

DOI: https://doi.org/10.3803/EnM.2012.27.4.323

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

Chang Jun Park, Joo Wan Seo, Hyeog Gyu Seoung, Jung Hee Koh, Yong Jae Lee, Bo Hyun Kim, In Ju Kim

Department of Internal Medicine, Pusan National University School of Medicine, Busan, Korea.

Corresponding author: Bo Hyun Kim. Division of Endocrinology and Metabolism, Department of Internal Medicine, Pusan National University Hospital, Pusan National University School of Medicine, 179 Gudeok-ro, Seo-gu, Busan 602-739, Korea. Tel: +82-51-240-7678, Fax: +82-51-254-3217, pons71@hanmail.net

Received 10 May 2012       Accepted 14 June 2012

Copyright © 2012 Korean Endocrine Society

(open-access):

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

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.

Keywords: Adrenocortical adenoma, Cushing syndrome, Pheochromocytoma

Figures and Tables

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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Table 1
Twenty-four hours urine test
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Table 2
Low dose dexamethasone suppression test
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17-OHCS, 17-hydroxycorticosteroids.

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Jung Hee Koh
https://orcid.org/http://orcid.org/0000-0002-6617-1449

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