Medulloblastom

Özet

Medulloblastom, özellikle çocuklarda posterior fossada görülen embriyonal tümördür ve sıkça vermis ya da orta hat serebellumdan kaynaklanır. Kontrastsız BT, posterior fossadaki vermis kitlesini ve yüksek dansiteli bileşenini gösterebilir. MR, tüm beyin ve omurilik incelemesiyle tümörün yerleşim ve yayılımını değerlendirmede kullanılır; solid metastazlar ve leptomeningeal yayılımın saptanmasında duyarlıdır. Baş ağrısı, bulantı-kusma ve ataksi medulloblastomda sık görülen belirtilerdir; posterior fossa tümörlerinde bilişsel bozukluk hidrosefaliyle ilişkili olabilir.

Faz ve pencere

Kontrastsız tanısal

Tümörün yerleşimi ve yayılımı için tüm beyin ve omuriliğin MR ile görüntülenmesi standarttır; ileri MR teknikleri hücresellik gibi özellikler hakkında ek bilgi sağlar.

BT bulguları

• Vermis veya orta hat serebellar kitle — çocukta posterior fossanın orta hattında yoğun yumuşak doku kitlesi olarak belirir.
• Yüksek hücreliliği nedeniyle tümör dokusu diffüzyon ağırlıklı görüntüleme (DWI) üzerinde kısıtlama gösterir ve ADC değerleri normal beyin dokusuna göre belirgin şekilde düşüktür.
• Dördüncü ventrikülün öne itilmesi veya silinmesi — kitle ventrikülü daraltır ve BOS geçişini engelleyebilir.
• Obstrüktif hidrosefali — lateral ve üçüncü ventriküller genişler; akut olguda periventriküler ödem de görülebilir.
• Kistik değişiklik veya nekroz — özellikle büyük kitlelerde heterojen düşük dansiteli iç alanlar bulunabilir.
• Tümör içi kalsifikasyon — mevcut olabilir, ancak tek başına tanımlayıcı özellik değildir.
• Beyin sapı basısı — posterior fossa kitlesi beyin sapına bası yapabilir.
• Medulloblastom BOS yoluyla kraniyal ve spinal leptomeninkslere yayılabilir; kontrastlı beyin ve tüm omurilik MRG’sinde subaraknoid ‘drop’ metastazları aranır.

Ölçütler ve sınıflamalar

WHO CNS5 medulloblastom sınıflaması

WHO CNS5’te medulloblastomlar WNT-aktive, SHH-aktive/TP53-yabanıl tip, SHH-aktive/TP53-mutant ve non-WNT/non-SHH olarak moleküler gruplara ayrılır. Risk sınıflaması cerrahi sonrası hastalık veya rezidü, yayılım, histoloji ve WNT alt grubu ile MYC durumu gibi moleküler özellikleri dikkate alır.

Normalde

Posterior fossa kitlesinin dansitesi, normal beyin parankiminin dansitesiyle karşılaştırılabilir. Şüpheli kitlenin dansitesini normal beyin parankimiyle karşılaştırın.

Ayırıcı tanı

Ependimom

dördüncü ventrikül içinde merkezlenir, ventrikülü genişletebilir ve çıkış foramenlerinden ince uzanım gösterebilir.

Pilositik astrositom

çocuklarda orta hat yerleşimi ergenlere göre daha sık görülebilir.

Atipik teratoid/rabdoid tümör

daha küçük çocukta posterior fossa kitlesi olabilir; BT görünümü örtüşür ve yaş tek başına tanı koydurmaz.

Beyin sapı gliomu

ponsu merkezden genişletir; dördüncü ventrikül kitlesinden farklı olarak pons dokusunun kendisi büyür.

Serebellar metastaz

özellikle erişkinde, bilinen primer tümör varlığında düşünülür; çoğu kez çevresel ödem ve birden fazla odak eşlik eder.

Tuzaklar

• Posterior fossa kitlesinde BT bulguları tanıya ipucu sağlayabilir; ileri MR teknikleri farklı tümör tiplerini ayırt etmeye yardımcı olur.
• Dördüncü ventrikülün küçük görünmesini tek başına kitle olarak yorumlama; vermis, pons ve ventrikül konturlarını kesitler boyunca takip et.

Kendini dene

1. Medulloblastom tipik olarak hangi bölgede ortaya çıkar?

   • Posterior fossada, serebellar vermis çevresinde
   • Frontal lobda
   • Hipofiz bezinde
   • Omuriliğin torakal bölümünde
   Cevabı göster

   Posterior fossada, serebellar vermis çevresinde. Medulloblastom tipik olarak posterior fossada, serebellar vermis çevresinde ortaya çıkar.

2. Lateral yerleşim medulloblastomda erişkin ve pediatrik olgularda nasıl karşılaştırılmıştır?

   • Erişkinlerde %57,6, pediatrik olgularda %14,4 bildirilmiştir.
   • Pediatrik olguların tamamında, erişkinlerin hiçbirinde lateral yerleşim görülmez.
   • Her iki yaş grubunda lateral yerleşim oranı aynıdır.
   • Lateral yerleşim yalnızca pediatrik olgularda bildirilmiştir.
   Cevabı göster

   Erişkinlerde %57,6, pediatrik olgularda %14,4 bildirilmiştir.. Meta-analizde lateral yerleşim erişkin olgularda %57,6, pediatrik olgularda %14,4 bildirilmiştir.

Kaynaklar

Bu sayfadaki 34 cümle ve 2 quiz sorusu aşağıdaki kaynaklardan alıntılarla otomatik olarak karşılaştırıldı; 18 cümle bu karşılaştırmada düzeltildi (2026-10-09).

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Eğitim amaçlıdır; hasta başında tanı ve tedavi kararının yerine geçmez, tıbbi tavsiye değildir. Metinler yapay zekâ desteğiyle yazıldı ve otomatik bir adımda kaynak alıntılarıyla karşılaştırıldı; bu bir tıbbi doğrulama değildir ve içerik uzman radyolog incelemesinden geçmedi. Klinik kararlarda güncel kılavuzları, kurum protokollerini ve radyoloji raporunu esas alın.