Pheochromocytoma due to unilateral adrenal medullary hyperplasia

Ghali Qupty, Avraham Ishay, Hava Peretz, Muralee Dharan, Nathan Kaufman, Rafael Luboshitzky*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review


We describe two male patients, aged 17 and 47 years, with clinical and biochemical features of pheochromocytoma. Both patients had normal-sized adrenal glands on abdominal CT scan and abnormal unilateral uptake of I-123 metaiodobenzylguanidine (MIBG) on scintigraphy. The surgical adrenalectomy revealed normal macroscopic glands in both patients. Histological examination showed adrenal medullary hyperplasia with adrenal cortico-medullary ratios of 2:1 and 4:1. Unilateral adrenalectomy resulted in amelioration of symptoms and normalization of catecholamines excretion. DNA examination for RET protooncogene revealed no mutations in exons 10, 11, 13, 14 and 16. Our results suggest that diffuse adrenal medullary hyperplasia may be the initial pathological change in the adrenal gland leading, subsequently, to the development of nodular hyperplasia and adrenal medullary tumor. These results indicate that the syndrome of pheochromocytoma may occur as an unilateral adrenal medullary hyperplasia in patients without evidence for multiple endocrine neoplasia.

Original languageEnglish
Pages (from-to)613-617
Number of pages5
JournalClinical Endocrinology
Issue number5
StatePublished - 1997
Externally publishedYes


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