Endocrinol Metab.  2011 Sep;26(3):258-262. 10.3803/EnM.2011.26.3.258.

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

• Jung JY
• Ahn SJ
• Yoon HY
• Ko JM
• Chung YS

• KMID: 2169179
• DOI: http://doi.org/10.3803/EnM.2011.26.3.258

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%.

Keyword

Neurofibromatosis type I; Pheochromocytoma; Prevalence

MeSH Terms

Humans
Hypertension
International Classification of Diseases
Korea
Medical Records
Neurofibromatoses
Neurofibromatosis 1
Pheochromocytoma
Prevalence

Figure

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  Fig. 1 Multiple skin neurofibromas with freckles and café-au-lait spots.

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  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.

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