Adrenokortikal Karsinomlar
Özet
Adrenokortikal karsinom (AKK), dünya genelinde milyonda 0,5–2 insidansla gözlenen, nadir ve agresif seyirli bir malignitedir. Kadınlarda daha sık rastlanan tümör, 5 yaş altı ve 40–60 yaş aralığında iki modlu yaş dağılımı sergiler. Klinik olarak cushing sendromu, virilizasyon gibi hormonal semptomlarla, lokal kitle ağrılarıyla veya tesadüfi bulgularla saptanır. Tanı aşamasında ENSAT protokollerine uygun hormonal değerlendirmeler, BT, MRG ve PET gibi radyolojik görüntülemeler ile patolojik Weiss kriterleri kullanılır. Evre 1 hastalıklarda 5 yıllık sağkalım %82 iken, metastatik evre 4 düzeyinde bu oran %13'e geriler. Tedavinin temelini tam cerrahi rezeksiyon oluşturur; nüks riski yüksek vakalarda ise adjuvan mitotan monoterapisi veya sisplatin bazlı kemoterapiler uygulanır. İleri evre, cerrahiye uygun olmayan olgularda EDP (etoposid, doksorubisin, sisplatin) artı mitotan kombinasyonu birinci basamak tedavi protokolüdür. Sonuç olarak, AKK prognozu düşük, yönetiminde multidisipliner yaklaşımlar ve prospektif moleküler araştırmalar gerektiren kompleks bir hastalıktır.
Adrenocortical carcinoma (ACC) is a rare and aggressive malignancy with a global incidence of 0.5–2 per million. Showing a bimodal age distribution predominantly affecting children under 5 and adults aged 40–60, it is more common in females. Clinically, ACC presents via hormonal excess symptoms like Cushing's syndrome or virilization, non-specific local pain, or incidental radiological findings. Diagnosis relies on comprehensive hormonal evaluations adhering to ENSAT guidelines, imaging modalities including CT, MRI, and PET, and pathological review using the Weiss criteria. Prognosis depends significantly on disease stage and resectability; the 5-year survival rate drops from 82% in stage 1 to 13% in stage 4. Complete surgical resection remains the only curative approach. For patients with high recurrence risks, adjuvant mitotane monotherapy or cisplatin-based chemotherapy is recommended. In advanced, unresectable cases, the combination of EDP (etoposide, doxorubicin, cisplatin) plus mitotane serves as the first-line standard regimen. Ultimately, ACC carries a poor prognosis, emphasizing the need for advanced prospective studies and molecular profiling to optimize therapeutic outcomes.
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