Open AccessAdrenocorticotropic hormone-producing pheochromocytoma: analysis of clinical cases
Author(s) -
Е И Марова,
Марова Евгения Ивановна,
И А Воронкова,
Воронкова Ия Александровна,
Anastasia Lapshina,
Лапшина Анастасия Михайловна,
Svetlana Arapova,
Арапова Светлана Дмитриевна,
Н С Кузнецов,
Кузнецов Николай Сергеевич,
L Ya Rozhinskaya,
Рожинская Людмила Яковлевна,
В А Животов,
Животов Владимир Анатольевич,
Oleg Borisovich Zharkov,
Жарков Олег Борисович,
Л. Е. Гуревич,
Гуревич Лариса Евсеевна,
G. A. Polyakova,
Полякова Галина Александровна
Publication year - 2015
Publication title -
ožirenie i metabolizm
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.154
H-Index - 5
eISSN - 2306-5524
pISSN - 2071-8713
DOI - 10.14341/omet2015346-52
Subject(s) - medicine , pheochromocytoma , metanephrines , adrenocorticotropic hormone , endocrinology , cushing syndrome , pathology , hormone
Ectopic secretion of ACTH from non-pituitary tumors, referred to as ectopic ACTH syndrome (EAS), accounts for about 10–20% of Cushing’s syndrome (CS). Ectopic hormone-secreting pheochromocytomas (Pheo) are rare. The first publication of association between pheochromocytoma and Cushing’s syndrome by Roux is dated 1955. Pheochromocytoma represents a rare cause of hypercortisolism, accounting for less than 5 % of ectopic Cushing’s syndrome while less than 1 % of pheochromocytomas is accompanied by Cushing’s syndrome.We are reporting 4 cases of ACTH-secreting pheochromocytoma presenting as Cushing’s syndrome. Data from 4 patients were analysed. There were 4 women from 50 to 63 years old. All patients had a clinical presentation of hypercorticoidism. Their levels of adrenocorticotropic hormone in plasma, 24-hour urinary free cortisol and urinary catecholamine were high. Computed tomography scan of the abdomen in all cases revealed a mass in the left adrenal gland. Left sided adrenalectomy was performed under treatment with a-blocker doxazosin and b-blocker atenolol. Histological examination revealed in 3 cases – pheocromocytoma and in 1 case corticomedullary mixed tumor of the adrenal gland. Additional immunostaining (IHC)of these tumors showed positive immunostaining for chromogranin and ACTH. The IHC search for somatostatin receptors of subtype 2 and 5 (SSTR2, SSTR5) was performed in 3 cases and showed predominately expression SSTR2. The case index of Ki-67 ranged, from 0,5 to 4%. Biochemical signs of hypercortisolism rapidly began to disappear after surgery. Follow up of the patients during the next 2 years on average was with disease remission.