ISSN: 1301-2193 E-ISSN: 1308-9846
  • Turkish Journal of
    Endocrinology and Metabolism

Adrenocorticotropic Hormon-Secreting Pheochromocytoma: A Rare Cause of Cushing’s Syndrome
Adrenokortikotropik Hormon Salgılayan Feokromositoma: Cushing Sendromunun Nadir Bir Nedeni
Department of Internal Medicine, Division of Endocrinology and Metabolism, Gazi University Faculty of Medicine, Ankara, Turkey *Department of Internal Medicine, Gazi University Faculty of Medicine, Ankara, Turkey **Department of General Surgery, Gazi University Faculty of Medicine, Ankara, Turkey ***Department of Pathology, Gazi University Faculty of Medicine, Ankara, Turkey ****Division of Endocrinology and Metabolism, Yüksek Ihtisas University, Ankara, Turkey
Doi: 10.25179/tjem.2018-63374 - Makale Dili: EN
Turk J Endocrinol Metab 2019;23:125-129
ABSTRACT
Pheochromocytoma is a rare cause of ectopic Cushing’s syndrome and presents a challenge to the clinician in the diagnosis and treatment. In this study, a rare case of adrenocorticotropic hormone-producing pheochromocytoma is presented. A 55-years-old man with the symptoms of hypercortisolism, multidrug-resistant hypertension, and an adrenal mass is presented. The laboratory results were consistent with hypercortisolism, high plasma adrenocorticotropic hormone, high 24-hour urine metanephrine and normetanephrine and severe hypokalemia. Abdominal computerized tomography showed a 3-cm non-adenoma left adrenal mass. After preoperative management, left adrenalectomy was performed. A histopathological examination revealed a 2.5 cm pheochromocytoma with focal positivity for adrenocorticotropic hormone. The patient was discharged with full recovery under hydrocortisone replacement therapy. Furthermore, six weeks after the operation, 24-hour urinary excretion of metanephrine and normetanephrine was within normal ranges. In conclusion, an adrenocorticotropic hormone-producing pheochromocytoma should be considered in patients with clinical manifestations of ectopic Cushing’s syndrome and adrenal mass.
ÖZET
Feokromositoma ektopik Cushing senrfomu’nun nadir bir nedenidir, tanı ve tedavi aşamalarında klinisyen için güçlük oluşturmaktadır. Çalışmamızda, nadir görülen adrenokortikotropik hormon salgılayan feokromositomalı bir hastanın sunulması amaçlanmıştır. Elli beş yaşında erkek hasta; hiperkortizolizm semptomları, dirençli hipertansiyon ve adrenal kitle nedeniyle kliniğimize kabul edildi. Laboratuvar bulguları hiperkortizolizmi destekler nitelikte idi. Yüksek plazma adrenokortikotropik hormondüzeyleri, artmış 24- saatlik idrar metanefrin ve normetanefrin düzeyleri ve ciddi hipokalemi olduğu görüldü. Abdomen bilgisayarlı tomografisinde sol adrenal bezde 3 cm boyutunda non-adenomatöz lezyon saptandı. Preoperatif hazırlık sonrası, sol adrenalektomi operasyonu yapıldı. Histopatolojik incelemede adrenokortikotropik hormon ile fokal boyanma gösteren 2,5 cm boyutunda feokromositoma rapor edildi. Operasyon sonrası hidrokortizon tedavisi ile taburcu edildi. Operasyondan altı hafta sonra, 24-saatlik idrar metanefrin ve normetanefrin düzeylerinin normal aralıkta olduğu görüldü. Sonuç olarak, adrenokortikotropik hormon-üreten feokromositoma ektopik Cushing sendromu düşünülen ve adrenal kitlesi olan hastalarda akılda bulundurulması gereken bir tanıdır.
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