Infantiles Hepatisches Hämangiom

◆Diagnostische Schlüsselhinweise

• 1US: Hypoechogene oder heterogene hepatische Masse mit erhöhter Doppler-Vaskularität — häufigste hepatische Masse im Säuglingsalter
• 2MRT: T2-hyperintense Masse mit früher peripherer und progressiver zentripetaler Anreicherung
• 3Proliferations- (0-12 Monate) und Involutionsphasen (1-5 Jahre)
• 4Diffuser Typ: High-Output-Herzversagen und konsumptive Hypothyreose — lebensbedrohlich
• 5GLUT1-Positivität — IHH-spezifisch

♦Pathophysiologie

Differentialdiagnostische Tipps

• →Hepatoblastom: häufigster pädiatrischer maligner Lebertumor, erhöhtes AFP, heterogene Kontrastierung, Kalzifikation
• →Mesenchymales Hamartom: zystisch dominant, stromale Komponente, normales AFP
• →Metastatisches Neuroblastom: adrenaler Primärtumor, erhöhte Katecholamine, ausgedehnte Metastasen
• →Hämangiom vom Erwachsenentyp: GLUT1-negativ, involutiert nicht, andere Biologie

●Klinische Bedeutung

Der häufigste Lebertumor bei Neugeborenen, und die überwiegende Mehrheit ist benigne. Jedoch können bei großen Läsionen lebensbedrohliche Komplikationen auftreten: Hochleistungsherzinsuffizienz (AV-Shunting), Kasabach-Merritt-Syndrom, abdominelles Kompartmentsyndrom. Hypothyreose sollte beim diffusen Typ gescreent werden (TSH). GLUT1-Positivität unterscheidet infantile Hämangiome von kongenitalen Hämangiomen (RICH/NICH). Propranolol hat die Behandlung revolutioniert. AFP-Monitoring ist wichtig zum Ausschluss eines Hepatoblastoms. Die meisten Fälle zeigen spontane Involution und erfordern keine Operation.

Referenzen

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• 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.
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