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Case Reports
. 2012 Jul;83(1):65-8.
doi: 10.4174/jkss.2012.83.1.65. Epub 2012 Jun 26.

Extra-adrenal pheochromocytoma after operation of congenital heart disease: a case report of 18-year-old boy

Bo-Hyun Hwang  1 , Hyun-Young Kim, Sung-Eun Jung, Kwi-Won Park
Affiliations
Case Reports

Extra-adrenal pheochromocytoma after operation of congenital heart disease: a case report of 18-year-old boy

Bo-Hyun Hwang et al. J Korean Surg Soc. 2012 Jul.

Abstract

Extra-adrenal pheochromocytoma is rare and presents variable symptoms. Its difficulty to diagnosis delays appropriate treatment. We would like to report an unusual case of extra-adrenal pheochromocytoma. The patient came to the emergency room with dyspnea, palpitation, and cyanosis. He had a history of hospitalization for Fontan operation due to congenital heart disease. Despite medication, his blood pressure remained high. After additional laboratory and image exams, he was diagnosed with extra-adrenal pheochromocytoma and had surgical treatment. The final pathology report was extra-adrenal pheochromocytoma with high risk of malignancy. The postoperative course was uneventful and showed normal laboratory results even after 3 months of outpatient follow-up. Extra-adrenal pheochromocytoma presents variable symptoms. We should consider endocrinologic diseases like extra-adrenal pheochromocytoma in cases presenting with palpitation and high blood pressure, even with a past history of cardiac surgery.

Keywords: Congenital heart defect; Fontan procedure; Paraganglioma; Pheochromocytoma.

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Conflict of interest statement

No potential conflict of interest relevant to this article was reported.

Figures

Fig. 1
Fig. 1
Para-aortic mass (arrow) is well defined on computed tomography (A) and metaiodobenzylguanidine test (B).
Fig. 2
Fig. 2
The permanent pathology reported the tumor as extra-adrenal pheochromocytoma, with a high risk of malignancy.
Fig. 3
Fig. 3
Microscopic findings of extra-adrenal pheochromocytoma. The trabecular arrangement (Zellballen) of neoplastic chief cells with abundant cytoplasm is evident (H&E, ×200).
See this image and copyright information in PMC

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