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Pankreas · Patoloji · Yüksek öncelik

Pankreatik nöroendokrin karsinom (PanNEC)

Pancreatic neuroendocrine carcinoma

Pankreatik nöroendokrin karsinom (PanNEC): yayımlanmış olgu görüntüsü, aksiyel kesit
Görüntü konuyu kısmen gösteriyor; bulgunun bir bölümü seçilebilir.

4 adım

Görüntü: Ohn JH, Kim YG, Lee SH ve ark., “Transformation of nonfunctioning pancreatic neuroendocrine carcinoma cells into insulin producing cells after treatment with sunitinib.”, 2013, Fig. 1. PMC3811707 · doi:10.3803/enm.2013.28.2.149 · CC BY-NC 4.0. Değişiklik: yeniden boyutlandırıldı.

Özet

PanNEC, küçük hücreli veya büyük hücreli morfoloji gösterebilen, kötü diferansiye ve yüksek dereceli pankreatik nöroendokrin neoplazmdır. İyi diferansiye PanNET G3 de yüksek proliferasyon gösterebilir, ancak PanNEC ile aynı hastalık değildir. Kontrastlı çok fazlı BT primer kitlenin yerini, damar ilişkisini ve karaciğer başta olmak üzere metastatik yayılımı haritalar; PanNEC çoğu zaman klasik hipervasküler PanNET görünümünü vermez. Hızlı büyüyen, nekrotik ve invaziv kitle ile yaygın metastaz tanıyı düşündürür, ancak kesin sınıflama doku örneği gerektirir. Lezyonun PanNET sanılması biyolojik davranış ve evrelemenin yanlış yorumlanmasına yol açabilir.

Faz ve pencere

Arteriyel tanısal
Primer kitle sıklıkla pankreas parankiminden daha az kontrastlanan heterojen solid kitle olarak öne çıkar; arteriyel seri tümör sınırını ve değişken ya da hipovasküler olabilecek karaciğer metastazlarını gösterir, ancak belirgin hipervasküler görünüm beklenmez.
Portal tanısal
Heterojen kitle, nekrotik düşük atenüasyonlu alanlar, karaciğer metastazları, bölgesel lenf nodları ve portal-splenik-mezenterik damarlarla temas veya invazyon değerlendirilir.
Kontrastsız
Kitle çoğunlukla izodens veya hafif hipodens yumuşak doku kitlesidir; kanama ya da kalsifikasyon varsa bu seri ek bilgi sağlar, fakat tek başına karakterizasyon için yeterli değildir.

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

BT bulguları

  • Pankreas kuyruğu veya gövdesinde büyük, düzensiz sınırlı, heterojen solid kitle; baş yerleşimi de mümkündür
  • İyi diferansiye PanNET'ten farklı olarak hipovasküler veya değişken kontrastlanan primer lezyon
  • Kitle merkezinde nekroz ya da kistik dejenerasyon biçiminde düşük atenüasyonlu alanlar
  • Pankreas konturunun bozulması ve komşu yağ planlarının silinmesiyle infiltratif büyüme
  • Yaygın venöz tromboz (splenik ve portal ven gibi) yapabilir; ayrıca komşu büyük damarlara ve organlara direkt invazyon ileri evre hastalığına işaret edebilir.
  • Çok odaklı veya çevresel kontrastlanan karaciğer metastazları; görünürlükleri kontrast fazına göre değişebilir
  • Peripankreatik ve üst abdominal lenf nodları ile uzak organ metastazları
  • Ana pankreatik kanal veya safra kanalında genişleme eşlik edebilir, fakat belirgin kanal genişlemesi şart değildir

Ölçütler ve sınıflamalar

WHO 2019: pankreatik nöroendokrin neoplazmlar
İyi diferansiye PanNET’ler G1–G3 olarak derecelendirilir; kötü diferansiye nöroendokrin karsinomlar küçük hücreli veya büyük hücreli morfolojide, yüksek dereceli tümörlerdir. İyi diferansiye PanNET G3, PanNEC ile eş anlamlı değildir; görüntüleme diferansiyasyonu kesinleştiremez.
AJCC Version 9: PanNET ve PanNEC evreleme kapsamı
AJCC Version 9 pankreatik NET protokolü iyi diferansiye PanNET evrelemesine aittir. Kötü diferansiye PanNEC ve MiNEN, organ-özgül karsinom ölçütleriyle evrelenir; PanNEC’ye PanNET protokolü uygulanmamalıdır.

Normalde

Normal pankreas parankimi homojen yapıda ve düzgün konturludur; ana pankreatik kanal belirgin genişleme veya kesinti göstermeden kesintisiz izlenir. Aynı düzeyde lezyonun parankime göre kontrastlanma farkını, kontur kaybını ve yağ planlarının korunup korunmadığını kıyasla; karaciğer segmentlerinde yeni odak veya damar lümeninde daralma normal görünümde bulunmaz.

Normal BT ile kıyasla

Aynı bölgenin, kaynak veri setinde patoloji içermediği belirtilen bir BT incelemesini kesit kesit kaydırın; organ sınırlarını açarak anatomiyi hasta görüntüsüyle karşılaştırın. Küçük rastlantısal bulgular tümüyle dışlanmamıştır.

Kaynak: TotalSegmentator v2.01 veri seti (Wasserthal ve ark., Radiology: Artificial Intelligence 2023), CC BY 4.0, vaka s0541; organ sınırları veri setinin otomatik segmentasyonundan, birleştirilerek sadeleştirildi. Görüntüler 2,5 mm kesit aralığına yeniden örneklendi.

Ayırıcı tanı

İyi diferansiye PanNET
sıklıkla sınırlı ve arteriyel fazda belirgin hipervaskülerdir; kötü diferansiye PanNEC daha sık heterojen, nekrotik ve infiltratiftir.
Pankreatik duktal adenokarsinom
çoğunlukla infiltratif hipovasküler kitle, ani kanal kesilmesi ve yukarı akım kanal genişlemesi/atrofi yapar; histoloji olmadan kesin ayrım yapılamaz.
Solid psödopapiller neoplazm
daha çok genç kadınlarda, iyi sınırlı kapsüllü kitle ve kanama-kistik dejenerasyonla görülür.
Pankreas metastazı
böbrek hücreli karsinom öyküsü ve arteriyel hipervasküler odaklar metastazı destekler; primer pankreatik NEC'de klinik öykü ve yayılım paterni ayırt ettirir.
Akut fokal pankreatit
çevresel inflamasyon ve sıvı değişiklikleri gösterebilir; kitle benzeri görünüm takipte gerileyebilir, ancak klinik ve doku değerlendirmesi önemlidir.

Tuzaklar

  • PanNEC ile iyi diferansiye PanNET G3’ü yalnız proliferasyon düzeyine göre eşitlemeyin; WHO sınıflaması diferansiyasyonu da esas alır ve ayrım histopatolojiktir.
  • PanNEC'yi hipervasküler görmeyi bekleme; kötü diferansiye olgular pankreas kitlesi ve karaciğer metastazlarında hipokontrastlanabilir.
  • Nekroz nedeniyle merkezdeki düşük atenüasyonu kistik neoplazm sanma; çevredeki solid komponenti ve infiltratif sınırı incele.
  • Bir fazda görünmeyen karaciğer odağını dışlama; metastaz kontrastlanması değişken olduğundan arteriyel ve portal serileri birlikte gözden geçir.

Kendini dene

  1. Pankreas kuyruğunda nekrotik, infiltratif kitle ve çok sayıda karaciğer odağı var; primer kitle belirgin hipervasküler değil. Hangi tümör tipi daha olasıdır?

    Cevabı göster

    Pankreatik nöroendokrin karsinom. Kötü diferansiye PanNEC heterojen, nekrotik ve infiltratif olabilir; hipervasküler PanNET görünümü şart değildir. İyi diferansiye PanNET çoğu kez sınırlı ve arteriyel hipervaskülerdir; seröz neoplazm mikrokistik, solid psödopapiller neoplazm ise genellikle kapsüllü ve dejeneratif görünür.

  2. Pankreatik kitleye ek olarak karaciğerde yeni odaklar görülüyor. Metastaz taramasında hangi BT incelemesi en uygundur?

    Cevabı göster

    Arteriyel ve portal fazlı pankreas protokolü. Pankreas protokolündeki arteriyel ve portal fazlar primer kitlenin damar ilişkisini ve değişken kontrastlanan karaciğer metastazlarını birlikte değerlendirmeye yardım eder. Kontrastsız, kemik ve üriner faz tek başına bu amaç için uygun değildir.

Kaynaklar

Bu sayfadaki 46 cümle ve 2 quiz sorusu aşağıdaki kaynaklardan alıntılarla tek tek karşılaştırıldı; 4 cümle bu karşılaştırmada düzeltildi (2026-10-08).

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