Adrenokortikal Karsinom
Özet
Adrenokortikal karsinom (ACC), adrenal bezinin korteksinden köken alan nadir ve agresif bir kanser olup kötü bir prognoza sahiptir. Metastatik hastalığı olan hastalarda 5 yıllık sağ kalım oranı %15'ten azdır. Bu bölümde, ACC'nin lokalize ve rezeke edilebilir formlardan ileri hastalığa kadar tanı ve tedavi seçeneklerinden bahsedip özellikle takip konusunda bilgi vereceğiz. ACC’nin nadir bir kanser olması nedeniyle takip prensipleri ile ilgili çalışmalar kısıtlıdır ve genellikle uzman tavsiyesi seviyesinde kalmaktadır. Genel olarak tedavi sonrası iki yıl boyunca her üç ayda bir, ardından beş yıl boyunca her altı ayda bir radyolojik ve hormonal açıdan takibi önerilmektedir. ACC karmaşık hormonal salgı sendromlarına yol açması ve mitotan gibi tedavisinde sık kullanılan ilaçların endokrinolojik sık ve karmaşık yan etkileri nedeniyle tedavi ve takibinde hormonal durumlar yakından izlenmeli ve bu açıdan endokrinoloji ile işbirliği yapılması önerilmektedir. Ayrıca nadir bir kanser olması ve özel cerrahi prensiplere gereksinim duyulması gibi durumlar göz önüne alındığında ACC konusunda özelleşmiş ileri merkezlerde tedavi ve takibi önerilmektedir. Hastalığın progresyonu açısından takibinde tedaviye tümör cevabı ve hormon salgılayan tümörlerde ilgili hormonun takibi önerilmektedir. Bunların dışında özellikle mitotan başta olmak üzere tedavide kullanılan ilaçların yan etkilerinin de belirli aralıklarla takibi gerekmektedir.
Adrenocortical carcinoma (ACC) is a rare and aggressive cancer of the adrenal cortex with a poor prognosis. The 5-year survival rate in patients with metastatic disease is less than 15%. In this section, we will discuss the diagnosis and treatment options for ACC, from localized and resectable forms to advanced disease, and provide information on follow-up care. Because ACC is a rare cancer, studies on follow-up principles are limited and generally remain at the level of expert advice. In general, radiologic and hormonal follow-up is recommended every three months for two years after treatment and then every six months for five years. Because ACC causes complex hormone secretion syndromes and because of the frequent and complex endocrinologic side effects of drugs commonly used in its treatment, such as mitotane, hormonal conditions should be closely monitored during treatment and follow-up, and collaboration with endocrinology is recommended in this regard. In addition, considering the fact that it is a rare cancer and that special surgical principles are required, treatment and follow-up are recommended in advanced centers specialized in ACC. In terms of disease progression, tumor response to treatment and monitoring of the relevant hormone in hormone-secreting tumors are recommended. In addition, the side effects of drugs used in treatment, especially mitotane, should be monitored at regular intervals.
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