Nadir Endometriyal Kanserler
Özet
Endometrium kanseri (EK), gelişmiş ülkelerde en sık görülen jinekolojik malignite olup, klasik olarak tip I ve tip II şeklinde sınıflandırılsa da, günümüzde moleküler özelliklerine göre POLE-ultra mutasyonlu, mikrosatellit instabilite (MSI)-hipermutasyonlu, gen kopya sayısı düşük (NSMP) ve gen kopya sayısı yüksek (p53abn) olmak üzere dört alt tipe ayrılmaktadır. Nadir görülen endometriyal kanserler, heterojen yapıları nedeniyle tanı, tedavi ve prognoz açısından zorluklar barındırır. Seröz karsinom, berrak hücreli karsinom, mikst karsinom, undiferansiye/dediferansiye karsinom ve karsinosarkom gibi yüksek dereceli ve agresif seyirli histolojik alt tipler, çoğunlukla p53abn veya MMRd moleküler profiliyle ilişkilidir ve nüks riskleri yüksektir. Ayrıca mezonefrik benzeri karsinom, gastrik tip müsinöz karsinom, trofoblastik/germ hücreli benzeri karsinom ve PEComa gibi son derece nadir görülen antiteler de mevcuttur. Bu nadir tümörlerin büyük kısmı, erken evrelerde bile uzak metastaz ve akciğer tutulumu yapabilen agresif klinik davranışlar sergilemektedir. Tedavinin belirlenmesinde hastalığın evresi en kritik faktör olmaya devam ederken, güncel ESGO/ESTRO/ESP kılavuzları nüks riskini belirlemek ve adjuvan tedavileri optimize etmek amacıyla moleküler sınıflandırma sistemlerini klinik yaklaşımlara entegre etmektedir.
Endometrial cancer (EC) is the most common gynecological malignancy in developed countries, traditionally classified into type I and type II; however, it is currently categorized into four molecular subtypes based on genomic architecture: POLE-ultramutated, microsatellite instability (MSI)-hypermutated, copy-number low (NSMP), and copy-number high (p53abn). Rare endometrial cancers pose significant diagnostic, therapeutic, and prognostic challenges due to their biological diversity. Aggressive histological variants, including serous, clear cell, mixed, undifferentiated/dedifferentiated carcinomas, and carcinosarcomas, are often associated with p53abn or MMRd profiles and carry a high risk of recurrence. Furthermore, extremely rare entities such as mesonephric-like, gastric-type mucinous, trophoblastic/germ cell-like carcinomas, and PEComas exhibit highly aggressive clinical behavior, often presenting with distant metastases even in early stages. While disease stage remains the most critical factor in determining treatment, contemporary ESGO/ESTRO/ESP guidelines integrate these molecular classifications into clinical practice to establish tailored risk groups and optimize adjuvant therapy protocols.
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