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Pheochromocytoma associated with cyanotic congenital heart disease

Korean Journal of Pediatrics 2008;51(1):93-97.
Published online January 15, 2008.
Pheochromocytoma associated with cyanotic congenital heart disease
Seung Joon Chung, Young Ah Lee, Choong Ho Shin, Sei Won Yang, Eun Jung Bae, Jung Il Noh
Department of Pediatrics, College of Medicine, Seoul National University, Seoul, Korea
청색증형 선천성 심질환에 동반된 갈색세포종
정승준, 이영아, 신충호, 양세원, 배은정, 노정일
서울대학교 의과대학 소아과학교실
Choong Ho Shin, Email: chshinpd@snu.ac.kr
Pheochromocytoma is a rare tumor of childhood, arising from adrenal medullary and chromaffin tissue. Because chronic hypoxia may induce pheochromocytoma, there have been several reports of pheochromocytoma development in cyanotic patients after corrective or palliative cardiac surgery. The variable clinical presentation of pheochromocytoma is obscured by both underlying heart disease and medications. If sudden hypertension, aggravation of a heart condition, or unusual symptoms such as diabetes mellitus develops in a cyanotic patient with congenital heart disease, pheochromocytoma must be ruled out. We report two patients presenting with cyanotic single-ventricle heart disease with pheochromocytoma.
Key Words: Pheochromocytoma, Cyanotic, Hypoxia, Congenital heart defect, Diabetes mellitus

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