Adrenal Adenomlar

Bilateral Adrenal Adenomlar
Bilateral Adrenal Adenomas
Nuri ÇAKIRa
aEndokrinoloji ve Metabolizma Hastalıkları BD, Gazi Üniversitesi Tıp Fakültesi, Emekli Öğretim Üyesi, Ankara, TÜRKİYE
Çakır N. Bilateral adrenal adenomlar. Güney E, editör. Adrenal Adenomlar. 1. Baskı. Ankara: Türkiye Klinikleri; 2019. p.59-63.
Article Language: TR
ÖZET
Bilateral adrenal kitleler, tesadüfen saptanan adrenal kitlelerin yaklaşık %15'ini oluşturmaktadır. Bunların daha sık görüleni, ACTH'dan bağımsız gelişen makronodüler adrenal hiperplazi (BMAH) dir. Bu adenomlar fazla kortizol salgısına neden olmaktadır. Bazı yazarlara göre, bilateral adrenal tümörü olan hastalarda, tek taraflı tümörü olan hastalara oranla, subklinik hiperkortizolemi daha sık görülmektedir. Subklinik hiperkortizolemi, klasik klinik Cushing sendromu belirtilerine neden olmaksızın, arteriyel hipertansiyon, glukoz intoleransı veya diyabet, hiperlipidemi, obezite ve osteoporoza neden olabilmektedir. Primer BMAH patogenezinde, adrenal dokuda lokal ACTH üretimine neden olan aberan hormon reseptörleri ve Armadillo repeat containing 5 (ARMC5) germline mutasyonu gibi gen mutasyonu saptanmıştır. BMAH'lı hastaların çoğunda, hafif kortizol fazlalığı vardır. Bu olguların değerlendirilmesi başlangıçta tek taraflı insidentalomalar gibidir, hormon fazlalığı özellikle kortizol fazlalığı (Cushing sendromu) aynı zamanda katekolamin ve aldosteron fazlalığı araştırılır. Tek taraflı adenomlardan farklı olarak, iki taraflı adenomu olan hastalarda özellikle adrenal yetmezlik ve konjenital adrenal hiperplazi dışlanmalıdır. Bütün hastalarda kortizol fazlalığı 1 mg deksametazon baskılama testi ile mutlaka dışlanmalıdır. Ciddi Cushing sendromu (idrar serbest kortizolünün normalin üç katından fazla olması) olan olgularda bilateral adrenalektomi önerilir. Kortizol fazlalığı klinik belirtileri olan ancak kortizol salgısı ılımlı artmış (idrar serbest kortizolünün normalin üç katından az olması) hastalarda tek taraflı adrenalektomi önerilmektedir. Avrupa Endokrin Derneği uzman komitesi, hastanın yaşı, genel durumu, birlikte olan diğer hastalıkları ve özellikle hastanın tercihi göz önünde tutularak seçilmiş hastalarda dominant nodüle adrenalektomi önermektedir.

Anahtar Kelimeler: Bilateral adenom; BMAH; subklinik Cushing; aberan GPCRs; ARMC5 gen mutasyonu
ABSTRACT
Bilateral masses constitute about 15% of incidentally discovered adrenal tumors. More frequently, these tumors are adenomas which are developed in the course of adrenocorticotropic hormone (ACTH)-independent macronodular adrenal hyperplasia (AIMAH), and, therefore, they may be a source of excessive cortisol secretion. According to some authors, a higher incidence of subclinical hypercortisolemia is found among patients with bilateral benign adrenal tumors than in those with unilateral tumors. Subclinical hypercortisolemia without clinically evident classic symptoms of Cushing syndrome may cause arterial hypertension, glucose intolerance or diabetes, hyperlipidemia, obesity and osteoporosis Aberrant hormone receptors, local production of corticotropin (ACTH) in adrenal tissues, and genetic mutations such a germline mutations of Armadillo repeat containing 5 gene (ARMC5) have been identified in the pathogenesis of primary BMAH. Most patients with BMAH present with bilateral incidentalomas and mild cortisol excess. Their initial evaluation is similar to that for a unilateral incidentaloma and includes tests to look for hormonal hypersecretion, most importantly, the degree of cortisol excess (Cushing's syndrome), but also catecholamine and aldosterone excess. In contrast to unilateral adenoma, adrenal insufficiency and congenital adrenal hyperplasia should be excluded specifically for patients with bilateral adenomas. Cortisol excess should be excluded in all patients with a 1 mg dexamethasone suppression test. Bilateral surgical adrenalectomy is recommended for patients with BMAH and severe Cushing's syndrome (elevated urinary free cortisol (UFC) >3 times above upper limit of normal). In patients with moderately increased cortisol production (less than threefold increase in UFC levels) but with clinical evidence of cortisol excess, unilateral adrenalectomy is recommended. The experts from the European Society suggested that in selected patients, a unilateral adrenalectomy of the dominant lesion may be considered based on age of the patients, degree of cortisol excess, general condition and comorbidities and very importantly patient preference.

Keywords: Bilateral adenomas; AIMAH; subclinic Cushing; aberran GPCRs; ARMC5 gene mutations

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