Adrenocorticotropic hormone-producing pheochromocytoma: analysis of clinical cases

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Abstract


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.


About the authors

Evgeniya Ivanovna Marova

Endocrinology Research Centre

Email: marova-e@mail.ru

Russian Federation professor, Sc.D., chief researcher at the Department of Neuroendocrinology and osteopathy,

Iya Aleksandrovna Voronkova

Endocrinology Research Centre

Author for correspondence.
Email: iya-v@yandex.ru

Russian Federation

Ph.D., assistant of laboratory histology, immunohistochemistry with a group of bio-banking

Anastasiya Mikhaylovna Lapshina

Endocrinology Research Centre

Email: nottoforget@yandex.ru

Russian Federation Ph.D., assistant of laboratory histology, immunohistochemistry with a group of bio-banking

Svetlana Dmitrievna Arapova

Endocrinology Research Centre

Email: arapova_s@inbox.ru

Russian Federation

Ph.D., a leading researcher at the Department of Neuroendocrinology and osteopathy

Nikolay Sergeevich Kuznetsov

Endocrinology Research Centre

Email: kuznetsov_enc@mail.ru

Russian Federation Professor, Sc.D., Head of the Surgery Department

Lyudmila Yakovlevna Rozhinskaya

Endocrinology Research Centre

Email: rozh@endocrincentr.ru

Russian Federation professor, Sc.D., chief researcher at the Department of Neuroendocrinology and osteopathy,

Vladimir Anatol'evich Zhivotov

Pirogov National Medical & Surgical Center

Email: jiv123@mail.ru

Russian Federation Ph.D., head of oncology, general and endocrine surgery department

Oleg Borisovich Zharkov

Pirogov National Medical & Surgical Center

Email: jarkov@mail.ru

Russian Federation Ph.D., head of the difficult diagnosis department

Larisa Evseevna Gurevich

Moscow Regional Research and Clinical Institute ("MONIKI")

Email: larisgur@mail.ru

Russian Federation Sc.D., professor, chief researcher at the Pathological Anatomy Department

Galina Aleksandrovna Polyakova

Moscow Regional Research and Clinical Institute ("MONIKI")

Email: polyakova@mail.ru

Russian Federation Sc.D., professor, chief researcher at the Pathological Anatomy Department

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Copyright (c) 2015 Марова Е.И., Воронкова И.А., Лапшина А.М., Арапова С.Д., Кузнецов Н.С., Рожинская Л.Я., Животов В.А., Жарков О.Б., Гуревич Л.Е., Полякова Г.А.

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