Von Hippel–Lindau sendromunda endokrin ilişkili BT örüntüsü

Özet

Von Hippel–Lindau (VHL) hastalığında adrenal feokromositoma, böbrek lezyonları ve pankreas kistleri/pankreatik NET aynı kalıtsal yatkınlığın parçaları olabilir. BT, mevcut incelemede adrenal kitlenin yerleşimini ve iki taraflılığını, ayrıca eşlik eden abdominal lezyonları değerlendirir; VHL'nin rutin abdominal taramasında MR tercih edilir. Arteriyel kontrastlanma hipervasküler odakları belirginleştirir, ancak görüntüleme tek başına VHL tanısı koymaz. Bir adrenal lezyonu izole adenom kabul etmek sendromik çoklu tümör yükünün gözden kaçmasına yol açabilir.

Faz ve pencere

Kontrastsız tanısal

Adrenal kitlenin bazal atenüasyonu, iç yağ, kanama ve kalsifikasyon değerlendirilir; feokromositoma çoğunlukla lipidten fakir görünür ve tek başına HU değeri sendromu tanımlamaz.

Arteriyel tanısal

Adrenal feokromositoma genellikle belirgin ve heterojen kontrastlanabilir; eşzamanlı hipervasküler böbrek veya karaciğer odağı araştırılır. Kontrastlanma paterni histolojiyi veya VHL genotipini tek başına kanıtlamaz.

Gecikmiş

Adrenal odak için washout ölçümleri alternatif tanılara yardımcı olabilir; feokromositomada washout adenoma benzeyebileceğinden bu ölçüm iki lezyonu güvenilir biçimde ayıramaz. Şüpheli feokromositoma biyokimyasal olarak plazma serbest veya idrar fraksiyone metanefrinlerle değerlendirilmelidir.

Önerilen pencereler: Batın (G 400 / M 50), Karaciğer (G 150 / M 30), Yumuşak doku (G 400 / M 40).

BT bulguları

• Adrenal medulladan köken alan kitle bir veya iki adrenal bezde görülebilir; VHL bağlamında karşı adrenal bez de dikkatle taranır.
• Feokromositoma kontrastsız BT'de lipidten fakir, kontrast sonrası belirgin ve heterojen olabilir; nekroz, kistik değişim veya kanama görünümü çeşitlendirir.
• Ekstra-adrenal paraganglioma retroperitoneal sempatik zincir boyunca hipervasküler kitle olarak görülebilir; VHL'de daha az tipiktir ve SDHx gibi diğer yatkınlıklar da düşünülür.
• Bilateral renal kitleler, renal kistler ve pankreas kistleri/pankreatik NET VHL'nin diğer abdominal ipuçlarıdır; bu konu sayfasında pankreas dışı odaklar değerlendirilir.
• Çoklu organlarda kistik ve solid lezyonların birlikteliği sendromik örüntüyü destekleyebilir; lezyonların her biri kendi organına göre karakterize edilmelidir.
• Karaciğer, kemik veya lenf nodunda metastaz kuşkusu varsa feokromositoma/paraganglioma yayılımı için ayrıca raporlanır.

Normalde

Normal adrenal bezler ince Y veya V biçimli kruslar halinde böbrek üst kutuplarını izler; belirgin nodül, genişleme ya da çevre yağda infiltrasyon beklenmez. VHL şüphesinde adrenal lezyonla birlikte renal kortikal kitleler de aranmalı; böbrek üst kutup komşuluğundaki nodülün kaynağı anatomik ilişkisi izlenerek değerlendirilmelidir.

Ayırıcı tanı

Adrenal adenom

lipid içeriği veya tipik washout destekleyebilir; feokromositoma da adenoma benzer washout gösterebilir.

Adrenokortikal karsinom

büyük, düzensiz, nekrotik kitle ve komşu invazyonu destekler.

Adrenal metastaz

bilinen primer tümör ve iki taraflı nodüller olasılığı artırır; VHL'de renal hücreli kanser öyküsü de sorgulanır.

Ekstra-adrenal paraganglioma

adrenal bezden ayrı, aort çevresi veya sempatik zincir yerleşimli yoğun kontrastlanan kitle.

Adrenal kanama

akut dönemde BT'de hiperdens hematom görünümündedir, takipte regresyon gösterir ve altta yatan tümörü maskeleyebilir.

Tuzaklar

• Feokromositoma washout ölçümü adenoma benzer çıkabilir; bu nedenle washout sonucu klinik ve sendrom öyküsünden bağımsız yorumlama.
• VHL'de adrenal feokromositoma görülebilir; adrenal bezden ayrı hipervasküler retroperitoneal kitleyi paraganglioma olasılığı açısından ayrıca değerlendirmek gerekir.
• Küçük renal kistler ve solid lezyonlar adrenal odağın dikkati çekmesiyle atlanabilir; iki böbreği ve karaciğeri ayrı ayrı tara.
• Tek bir BT'de tüm sendrom bulgularının görülmesi beklenmez; görüntüleme negatifliği genetik yatkınlığı dışlamaz.

Kendini dene

1. VHL öyküsü bulunan kişide iki adrenal bezde belirgin kontrastlanan kitleler görülüyor. En uygun görüntüleme yorumu hangisidir?

   • Bilateral feokromositoma olasılığı
   • Bilateral adrenal adenom
   • Adrenal miyelolipom
   • Adrenal kanama
   Cevabı göster

   Bilateral feokromositoma olasılığı. VHL bağlamında iki taraflı, belirgin kontrastlanan adrenal kitleler feokromositomayı düşündürür. Adenom lipid/washout özellikleriyle, miyelolipom makroskopik yağla, kanama ise kontrastsız yüksek atenüasyon ve iç kontrastlanma yokluğuyla ayrılır.

2. VHL tanılı hastada aort önünde adrenal bezden ayrı hipervasküler retroperitoneal kitle var. Bu yerleşim en çok neyi düşündürür?

   • Paraganglioma
   • Adrenal korteks adenomu
   • Renal angiomyolipoma
   • Duodenal divertikül
   Cevabı göster

   Paraganglioma. Adrenal bezden ayrı paraaortik hipervasküler kitle, ekstra-adrenal paraganglioma ile uyumlu bir yerleşim ve görünüm sergiler.

Kaynaklar

Bu sayfadaki 38 cümle ve 2 quiz sorusu aşağıdaki kaynaklardan alıntılarla tek tek karşılaştırıldı; 6 cümle bu karşılaştırmada düzeltildi (2026-10-09).

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