Türk Nöroşirürji Dergisi 2014 , Vol 24 , Num - Ek
Cushing’s Disease and Surgical Treatment
1İstanbul Üniversitesi, Cerrahpaşa Tıp Fakültesi, Nöroşirürji Anabilim Dalı, İstanbul, Türkiye
2Prof. Dr. Mazhar Osman Bakırköy Ruh Sağlığı Hastalıkları Eğitim ve Araştırma Hastanesi, Nöroşirürji Kliniği, İstanbul, Türkiye
3Ondokuz Mayıs Üniversitesi, Tıp Fakültesi, Nöroşirürji Anabilim Dalı, Samsun, Türkiye
The cause of more than 70% of endogenous Cushing syndrome cases is the excess secretion of adrenocorticotropic hormone (ACTH) from the hypophysis. Cushing’s disease is the result of excessive glucocorticoid and androgen secretion due to an ACTH-secreting pituitary adenoma. Approximately half of untreated Cushing’s disease patients will die within 5 years, necessitating total surgical resection of the tumor and hormonal control. An early indicator of total tumor resection and remission is the need for glucocorticoid replacement in the early postoperative period. Excision of the ACTH-secreting adenoma together with its pseudocapsule leads to rapid postoperative decrease of cortisol levels and increases the permanent remission rate. Repeat surgery should be considered if remission is not achieved surgically and residual tumor is present, and a second surgery should also be considered in case of a recurrence in the long term. Medical treatment is only used as adjuvant in Cushing’s disease. Repeat surgery can be used in cases without remission while hemihypophysectomy or total hypophysectomy can be considered in elderly patients. Radiotherapy can be used when pituitary surgery is not successful. Cushing’s disease patients should be under long-term follow-up due to the high recurrence rates.

Cushing syndrome (CS) develops due to chronic glucocorticoid excess. CS can be evaluated in two groups as spontaneous and iatrogenic. Spontaneous CS can also be divided into two as ACTH-dependent and ACTH-independent. The most common (70%) cause of spontaneous CS in adults is Cushing’s disease (CD) developing due to an adrenocorticorticotropic hormone (ACTH)-secreting pituitary adenoma. Bilateral adrenal cortical hyperplasia develops as a result of the ACTH secretion from the pituitary adenoma in CD and the excessive secretion of adrenal cortical hormones leads to clinical findings. The typical clinical findings of CD and the fact that the disorder is due to a basophilic adenoma were first defined by Harvey Cushing in 1932. CD is usually seen in adult females aged 25 to 45 years and the annual incidence is estimated as 2-3 per million. Ectopic ACTH secretion is much less common.

We discuss CS etiology, CD pathophysiology, the diagnostic approach, treatment options and especially the result of surgical treatment in this article. Anahtar Kelimeler : Cushing’s disease, Surgical treatment