MEN tip 2: feokromositoma örüntüsü

Özet

MEN2, RET ilişkili kalıtsal bir sendrom olup medüller tiroid karsinomu, feokromositoma ve bazı alt tiplerde paratiroid hastalığı örüntüsüyle tanınır. MEN2A’da adrenal feokromositoma görülebilir; BT, biyokimyasal olarak şüpheli lezyonun yerini ve yayılımını değerlendirmeye yardımcı olur. Bilateral veya çok odaklı adrenal kitle, kalıtsal yatkınlığı düşündürür ve tek bir sporadik adrenal kitle varsayımını sorgulatır. Tanınmamış feokromositoma ciddi hipertansif olaylarla ilişkili olabileceği için adrenal bulgunun ve diğer adrenal bezin atlanmaması klinik açıdan önemlidir.

Faz ve pencere

Kontrastsız

Feokromositoma çoğunlukla lipidten zengin adenom gibi düşük atenüasyon göstermez; heterojen yumuşak doku kitlesi, kanama, kalsifikasyon veya merkezî düşük atenüasyonlu alanlar görülebilir. Tek bir atenüasyon değeri tanıyı doğrulamaz.

Arteriyel tanısal

Adrenal medulla kökenli kitle belirgin ve erken kontrastlanabilir; nekrotik, kistik veya hemorajik bölümler canlı kontrastlanan dokudan ayrılır. Her iki adrenal bez bu fazda ayrı ayrı incelenir.

Portal

Lezyonun adrenal korteksle ilişkisi, çevre organlara uzanımı ve bölgesel nodlar değerlendirilir; kontrast yıkanması değişkendir ve adenom-feokromositoma ayrımı için tek başına güvenilir değildir.

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

BT bulguları

• Feokromositoma adrenal bez içinde merkezlenir; normal adrenal bacakların şeklini bozan, medulladan kaynaklanan yuvarlak ya da lobüle kitle olabilir.
• MEN2 örüntüsünde sağ ve sol adrenal bezler ayrı ayrı taranır; iki tarafta benzer veya farklı boyutta kitleler bulunabilir.
• Arteriyel fazda canlı tümör dokusu erken ve belirgin kontrastlanabilir; büyük lezyon heterojen görünüm kazanabilir.
• Merkezî nekroz, kistik değişim, kanama veya kalsifikasyon eşlik edebilir; bu bileşenler tüm kitleyi homojen solid kabul etmeyi engeller.
• Bilateral adrenal kitle veya bir tarafta kitleyle karşı adrenal bezde nodüler medulla görünümü kalıtsal sendrom olasılığını artırır.
• Adrenal dışı retroperitoneal ya da baş-boyun paragangliomları MEN2 ile ilişkili adrenal fenotipten farklı dağılım gösterir; görüntülenen alan kapsamına göre ayrıca taranır.

Ölçütler ve sınıflamalar

MEN2 moleküler tanısı

Klinik olarak düşündüren bulgularla birlikte germline RET patojenik/olası patojenik varyant

MEN2 klinik alt tipleri

MEN2A: MTC, feokromositoma ve primer hiperparatiroidizm kombinasyonu; MEN2B: MTC, feokromositoma, mukozal/intestinal ganglionomatoz ve marfanoid özellikler; FMTC: diğer MEN2 bulguları olmaksızın yalnızca ailesel MTC görülen fenotip.

Normalde

Normal adrenal bezler karaciğer ve vena kava komşuluğunda sağda, dalak ve böbrek üst kutbu komşuluğunda solda ince, dallanan yumuşak doku yapıları olarak izlenir; bez içinde ayrı kitle bulunmaz. Aynı aksiyel ve koronal kesitlerde her iki bezin kalınlığını, bacaklarının devamlılığını ve çevre yağ planını karşılaştır; kitleyi adrenal bezin parçası mı yoksa komşu bir retroperitoneal lezyon mu olarak izlediğini belirle.

Ayırıcı tanı

Adrenokortikal adenom

çoğunlukla kontrastsız seride lipid içeriğine bağlı düşük atenüasyon gösterir; feokromositomada atenüasyon ve washout değişken olabilir.

Adrenokortikal karsinom

büyük, düzensiz sınırlı kitle, komşu organ invazyonu, venöz tümör trombüsü veya uzak metastazla öne çıkabilir.

Adrenal metastaz

bilinen primer tümör, iki taraflı nodüller ve eşlik eden diğer metastaz alanları ayırıcı tanıyı destekler.

Adrenal kortikal hiperplazi

bezin genel kalınlaşması veya nodüler büyümesi şeklindedir; medulladan çıkan ayrı, yoğun boyanan kitle yapısı daha az tipiktir.

Adrenal kanama

kontrastsız seride yüksek atenüasyonlu içerik ve çevresel yağ dokuda reaksiyon olabilir; kontrast sonrası canlı tümör dokusu şeklinde nodüler boyanma beklenmez.

Tuzaklar

• Feokromositoma kontrastı adenom gibi hızlı yıkayabilir; washout değerlerini tek başına adenoma kanıt sayma.
• Küçük karşı taraf adrenal kitlesi ilk odak kadar belirgin olmayabilir; her iki bezi aksiyel ve koronal görüntülerde bağımsız taray.
• Yoğun kontrastlanan damar kesiti adrenal nodülü taklit edebilir; yapıyı komşu arter veya ven lümeniyle devamlılık açısından takip et.
• Nekrotik merkez tümörün canlı kenarını gizleyebilir; kontrastsız ve arteriyel kesitleri aynı anatomik seviyede karşılaştır.

Kendini dene

1. RET ilişkili sendromu olan hastada her iki adrenal bezde hipervasküler kitleler saptandı. En olası örüntü?

   • Bilateral feokromositoma
   • Adrenokortikal adenom
   • Adrenal miyelolipom
   • Adrenal kanama
   Cevabı göster

   Bilateral feokromositoma. MEN2'de adrenal medulladan kaynaklanan feokromositomalar bilateral olabilir; her iki bezde erken boyanan kitle bu örüntüyle uyumludur. Adenom çoğunlukla kortikal ve lipid içeriklidir; miyelolipom makroskopik yağ içerir, kanamada ise canlı hipervasküler kitle dokusu beklenmez.

2. Adrenal kitle kontrast sonrası hızlı washout gösteriyor. Bu bulgu feokromositomayı nasıl etkiler?

   • Dışlamaz; washout örtüşebilir
   • Kesin feokromositomadır
   • Kesin metastazdır
   • Kanamayı kanıtlar
   Cevabı göster

   Dışlamaz; washout örtüşebilir. Feokromositomalar adenoma benzer washout gösterebilir; washout tek başına ayırıcı değildir. Bulgular biyokimyasal değerlendirme ve lezyonun diğer BT özellikleriyle birlikte ele alınır.

İlgili konular

• MEN tip 1: hipofiz–duodenum/pankreas–adrenal örüntüsü
• Retroperitoneal paraganglioma
• Organ of Zuckerkandl ve pelvik paraganglioma

Kaynaklar

Bu sayfadaki 42 cümle ve 2 quiz sorusu aşağıdaki kaynaklardan alıntılarla tek tek karşılaştırıldı; 4 cümle bu karşılaştırmada düzeltildi (2026-10-08).

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