Malign Plevral Mezotelyoma
Özet
Malign Plevral Mezotelyoma (MPM), plevral boşluktaki mezotelyal yüzeylerden kaynaklanan, nadir ve agresif bir malign tümördür; epiteloid, sarkomatoid ve bifazik olmak üzere üç ana histolojik alt tipe ayrılmaktadır. Hastalığın epidemiyolojisinde uzun süreli mesleki veya ikincil asbest maruziyeti en temel risk faktörüyken, Türkiye’de Nevşehir Ürgüp bölgesinde volkanik eriyonit minerali içeren yalıtımlar da yüksek MPM vakalarıyla ilişkilendirilmiştir. Genetik çalışmalarda ise BAP1 germline ve somatik mutasyonlarının tümör gelişimi üzerindeki rolü gösterilmiştir. Tanı aşamasında metastatik adenokarsinomlardan ayırt edilmesinde PAS boyaması ile kalretinin, WT1, vimentin ve CK5/6 gibi immünohistokimyasal belirteçler kullanılır; benign ve malign ayrımında ise P16 delesyon kaybına bakılmaktadır. Evrelemede TNM sistemi kullanılmakta olup, cerrahi rezeksiyondan fayda görecek hastaları belirlemek adına PET-BT, mediastinoskopi ve EBUS gibi yöntemlerle kapsamlı değerlendirmeler yapılır. Ameliyata uygun hastalarda Plörektomi/Dekortikasyon (P/D) veya Ekstraplevral Pnömonektomi (EPP) yöntemleri sıkı preoperatif solunum ve kardiyak kriterler doğrultusunda tercih edilir. Rezeke edilemeyen epiteloid MPM vakalarında standart ilk basamak tedavi sisplatin ve pemetrekset kombinasyonuyken, non-epiteloid tipte nivolumab ve ipilimumab immünoterapi kombinasyonu FDA onaylı seçkin bir yaklaşımdır. Lokal hastalık kontrolünü artırmak amacıyla cerrahi sonrasında yoğunluk ayarlı radyoterapi (IMRT) gibi gelişmiş radyoterapi tekniklerinden yararlanılır ve progresyon durumunda sonraki basamak tedaviler platin duyarlılığına göre planlanır.
Malignant Pleural Mesothelioma (MPM) is a rare and aggressive malignant tumor originating from the mesothelial surfaces of the pleural cavity, classified into epithelioid, sarcomatoid, and biphasic histological subtypes. Long-term occupational or secondary exposure to asbestos is well-established as the primary epidemiological factor, while in Turkey's Nevşehir Ürgüp region, insulation with the volcanic mineral erionite is heavily linked to increased cases. Genetically, germline and somatic mutations in the BAP1 gene play a significant role in tumor development. In diagnosis, PAS staining along with immunohistochemical markers such as calretinin, WT1, vimentin, and CK5/6 are utilized to differentiate MPM from metastatic adenocarcinomas, whereas P16 deletion loss helps distinguish malignant from benign proliferations. Staging relies on the TNM system, where PET-CT, mediastinoscopy, and EBUS are performed to thoroughly evaluate resectability. For surgical candidates, Pleurectomy/Decortication (P/D) or Extrapleural Pneumonectomy (EPP) are preferred based on strict preoperative pulmonary and cardiac functional criteria. In unresectable disease, first-line standard therapy for the epithelioid subtype consists of cisplatin and pemetrexed, while the nivolumab and ipilimumab immunotherapy combination is the FDA-approved select option for non-epithelioid cases. To improve local disease control, advanced techniques like intensity-modulated radiotherapy (IMRT) are utilized post-surgery, and subsequent treatments upon progression are determined by platinum sensitivity.
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