Premium
Pheochromocytoma due to unilateral adrenal medullary hyperplasia
Author(s) -
Qupty Ghali,
Ishay Avraham,
Peretz Hava,
Dharan Muralee,
Kaufman Nathan,
Luboshitzky Rafael
Publication year - 1997
Publication title -
clinical endocrinology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.055
H-Index - 147
eISSN - 1365-2265
pISSN - 0300-0664
DOI - 10.1046/j.1365-2265.1997.2841134.x
Subject(s) - pheochromocytoma , medicine , hyperplasia , adrenalectomy , multiple endocrine neoplasia type 2 , medullary cavity , endocrinology , adrenal gland , adrenal medulla , scintigraphy , pathology , multiple endocrine neoplasia , catecholamine , biology , germline mutation , biochemistry , mutation , gene
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 proto‐oncogene 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.