Case Reports and Estimated Prevalence of Adrenal Pheochromocytoma in Patients with Neurofibromatosis Type I in Korea.

Jung, Ju Yang; Ahn, Seun Joo; Yoon, Hyung Young; Ko, Jung Min; Chung, Yoon Sok

doi:2011.26.3.258

Endocrinol Metab > Volume 26(3); 2011 > Article

Case Report

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.

Abstract

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