Malign Mediasten Hastalıkları
Özet
Malign mediasten hastalıkları, anterior, visseral ve paravertebral kompartmanlarda yerleşen ve mediastinal lezyonların yaklaşık %30’unu oluşturan patolojilerdir. Bu hastalıklar, çocuklarda %40-50, yetişkinlerde ise %25 oranında malignite göstermektedir. En sık anterior mediastende yerleşen lezyonlar arasında sırasıyla timoma, lenfoma, endokrin tümörler ve malign germ hücreli tümörler (GHT) yer alır. Germ hücreli tümörler matür/immatür teratomlar, seminomlar ve non-seminomatöz tümörler olarak sınıflandırılır. Tanı süreçlerinde akciğer grafileri, bilgisayarlı tomografi (BT), manyetik rezonans görüntüleme (MRG), endobronşiyal ultrasonografi (EBUS) ve PET-BT gibi görüntüleme yöntemlerinden yararlanılırken, ayırıcı tanıda serum tümör markerları (AFP, β-HCG) kritik öneme sahiptir. Visseral mediastende küçük hücreli veya küçük hücreli dışı akciğer kanserleri ile Castleman hastalığı görülürken; paravertebral alanda çoğunlukla benign sinir kılıfı tümörleri yer alsa da malign schwannomlar ve nöroblastomlar gibi nörojenik tümörler de saptanır. Tedavi yaklaşımı tümörün histolojisine göre değişmekte olup; matür teratomlarda komplet cerrahi eksizyon (R0 rezeksiyon) kür sağlarken, seminomlarda ilk basamak tedavi sisplatin bazlı kemoterapi ve radyoterapidir. Non-seminomatöz ve malign nörojenik tümörlerde ise multimodatite tedaviler uygulanmasına rağmen prognoz genellikle daha kötüdür.
Malign mediastinal diseases are pathologies located in the anterior, visceral, and paravertebral compartments, accounting for approximately 30% of mediastinal lesions. These diseases show a malignancy rate of 40-50% in children and 25% in adults. Lesions are most frequently located in the anterior mediastinum, including thymoma, lymphoma, endocrine tumors, and malignant germ cell tumors (GHT), respectively. Germ cell tumors are classified into mature/immature teratomas, seminomas, and non-seminomatous tumors. Diagnostic processes utilize chest X-rays, computed tomography (CT), magnetic resonance imaging (MRI), endobronchial ultrasound (EBUS), and PET-CT, while serum tumor markers (AFP, β-HCG) are critically important for differential diagnosis. While small cell or non-small cell lung cancers and Castleman disease are observed in the visceral mediastinum; neurogenic tumors such as malignant schwannomas and neuroblastomas can also be detected in the paravertebral area, although benign nerve sheath tumors predominate there. The treatment approach varies according to the histology of the tumor; complete surgical excision (R0 resection) provides a cure in mature teratomas, whereas the primary treatment in seminomas is cisplatin-based chemotherapy and radiotherapy. In non-seminomatous and malignant neurogenic tumors, the prognosis is generally worse despite multimodal treatments.
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