Endocrinol Metab > Volume 26(3); 2011 > Article
Endocrinology and Metabolism 2011;26(3):258-262.
DOI: https://doi.org/10.3803/EnM.2011.26.3.258    Published online September 1, 2011.
Case Reports and Estimated Prevalence of Adrenal Pheochromocytoma in Patients with Neurofibromatosis Type I in Korea.
Ju Yang Jung, Seun Joo Ahn, Hyung Young Yoon, Jung Min Ko, Yoon Sok Chung
1Department of Endocrinology and Metabolism, Ajou University School of Medicine, Suwon, Korea. yschung@ajou.ac.kr
2Department of Medical Genetics, Ajou University School of Medicine, Suwon, Korea.
We report three patients with adrenal pheochromocytoma who were associated with type I neurofibromatosis. Two of them were asymptomatic, but one case involved hypertension. We reviewed medical records and adrenal imaging, and estimated the prevalence of adrenal pheochromocytoma among neurofibromatosis type I patients in one university hospital in Korea. A total of 658 patients were coded for neurofibromatosis type I (Q85.0 with International Classification of Diseases 10 version) with clinical impression, but only 371 were confirmed via 1997 National Institute of Health criteria. Adrenal images were generated in 203 patients, and 3 of them were diagnosed with pheochromocytoma. According to the results of this study, the estimated prevalence of adrenal pheochromocytoma in type I neurofibromatosis was 0.30-1.48%.
Key Words: Neurofibromatosis type I, Pheochromocytoma, Prevalence

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