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Jung, Ahn, Yoon, Ko, and Chung: Case Reports and Estimated Prevalence of Adrenal Pheochromocytoma in Patients with Neurofibromatosis Type I in Korea
Case Report
Endocrinology and Metabolism

Endocrinology and Metabolism 2011; 26(3): 258-262.

Published online: 27 September 2011

DOI: https://doi.org/10.3803/EnM.2011.26.3.258

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 Ko1, Yoon-Sok Chung

Department of Endocrinology and Metabolism, Ajou University School of Medicine, Suwon, Korea.

1Department of Medical Genetics, Ajou University School of Medicine, Suwon, Korea.

Corresponding author: Yoon-Sok Chung. Department of Endocrinology and Metabolism, Ajou University School of Medicine, San 5 Wonchon-dong, Yeongtong-gu, Suwon 443-721, Korea. Tel: +82-31-219-5127, Fax: +82-31-219-4497, yschung@ajou.ac.kr

Received 4 August 2010       Accepted 9 January 2011

Copyright © 2011 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

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%.

Keywords: Neurofibromatosis type I, Pheochromocytoma, Prevalence

Figures and Tables

Fig. 1
Multiple skin neurofibromas with freckles and café-au-lait spots.
enm-26-258-g001
Fig. 2
A. MRI shows a 12-cm-sized right adrenal mass with heterogeneous enhancement. B. CT revealed a 12-cm-sized multi-cystic mass on the right adrenal gland. C. Gross pathology finding revealed adrenal pheochromocytoma composed by fibrous and adipose tissues with cystic change.
enm-26-258-g002
Table 1
Diagnostic criteria of neurofibromatosis type I (National Institute of Health, 1997)
enm-26-258-i001

*Two or more of the criteria should be met for diagnostic confirmation.

Table 2
Estimation of prevalence of adrenal pheochromocytoma in neurofibromatosis type I
enm-26-258-i002

NIH, National Institute of Health.

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