İnfantil Hepatik Hemanjiom

◆Ayırıcı Tanı İpuçları

• 1US'de hipoekoik veya heterojen hepatik kitle ile artmış Doppler vaskülarite — süt çocuğunda en sık hepatik kitle
• 2MR'da T2 hiperintens kitle ile erken periferik ve progresif santripetal (dıştan içe) kontrastlanma
• 3Proliferasyon (0-12 ay) ve involüsyon (1-5 yaş) fazları — boyut değişimi takipte görülür
• 4Diffüz tipte yüksek debili kalp yetmezliği ve konversiyon hipotiroidizmi — hayatı tehdit edici
• 5GLUT1 pozitifliği (immunohistokimya) — IHH'ye spesifik, diğer vasküler tümörlerden ayırt edici

♦Patofizyoloji

Diferansiyel İpuçları

• →Hepatoblastom: en sık pediatrik malign karaciğer tümörü, AFP yüksek, heterojen kontrastlanma, kalsifikasyon
• →Mezenkimal hamartom: kistik dominant, stromal komponent, AFP normal
• →Metastatik nöroblastom: adrenal primer, katekolamin yüksek, yaygın metastazlar
• →Erişkin tip hepatik hemanjiom: GLUT1 negatif, involüsyon göstermez, farklı biyoloji

●Klinik Önem

Yenidoğanların en sık karaciğer tümörüdür ve büyük çoğunluğu benigndir. Ancak büyük lezyonlarda hayatı tehdit eden komplikasyonlar gelişebilir: yüksek debili kalp yetmezliği (AV shunting), Kasabach-Merritt sendromu, abdominal kompartman sendromu. Diffüz tipte hipotiroidizm taranmalıdır (TSH). GLUT1 pozitifliği infantil hemanjiomları konjenital hemanjiomlardan (RICH/NICH) ayırır. Propranolol tedavide devrim yaratmıştır. AFP takibi hepatoblastom ekartasyonu için önemlidir. Çoğu olgu spontan involüsyon gösterir ve cerrahi gerektirmez.

Kaynaklar

• Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Christison-Lagay ER, et al. Hepatic hemangiomas: subtype classification and development of a clinical practice algorithm and registry. J Pediatr Surg. 2007;42(1):62-7.
• ISSVA classification for vascular anomalies (updated 2018). International Society for the Study of Vascular Anomalies.
• Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Huang SA, et al. Severe hypothyroidism caused by type 3 iodothyronine deiodinase in infantile hemangiomas. N Engl J Med. 2000;343(3):185-9.
• ACR Appropriateness Criteria: Liver Lesion in Infants and Children. 2019.
• Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Defined by Leauté-Labrèze C, et al. Propranolol for severe hemangiomas of infancy. N Engl J Med. 2008;358(24):2649-51.