Fritz Schneble, M. Ebert (Regensburg)  

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5 Months  

Male  

Abdomen-Liver  

neoplastic  

5 month-old boy. In the patient's history, multiple recurring infections (2 x respiratory infections, 1 x gastroenteritis). Obvious hepatomegaly, no further clinical findings. Labs (including blood count and coagulation) were without pathological findings. AFP and ß-HCG and catecholamine metabolites were in normal ranges.  

Benign neoplasia  

<- view Ultrasound 1

Ultrasound 1: Sonography of the liver in the upper abdominal transverse slice (1)
The multiple round and oval hypoechogenic structures are shown to be disseminated. These are mostly 1,5 -2cm in diameter. All liver segments are afflicted. The most focuses are shown in the region of the hepatic veins and the portal vessels. The rest of the abdomen/retroperitoneal space is unremarkable.




<- view Ultrasound 2

Ultrasound 2: In color doppler, the liver focuses show even in the highly-sensitive color doppler no increase of perfusion (in comparison to the liver parenchyma). Unremarkable flow in the celiac trunk, in the hepatic veins and in the vena portae.





<- view MRI 1

MRI 1: T2-weighted transversal: The right and left liver lobes are completely infiltrated with multiple round signal intense tumors. (1,0 - 2,5 cm Diameter).

In T1 - weighted images (not shown here), the changes are hypointens. They absorb massively the contrast media after given, and are in late-phases (except central contrast media exclusion in larger focuses) totally contrasted.



Supplement from 06/18/2004 (F. Schneble):





<- view Ultrasound 3

Ultrasound 3: (1 1/2 years later) Sonographically only 3 hypoechoic structures with max. 9 mm diameter shown.




<- view Ultrasound 4

Ultrasound 4: (1 1/2 years later) Within these structures no increased perfusion is shown.

 

Surgery / Histo  

Metastasis of a neuroblastoma, hepatoblastoma  

Manifestation as a solitary tumor or like in the described case as multiple focuses (also described as hemangioendotheliomatosis). The course is variable. Spontaneous remissions have been described. Findings and clinical signs can run in a progredient course. Severe complications can be a development of a Kasabach-Meritt syndrome but also cardial decompensation through a massive AV-shunt is possible.  

Histology: infantile hemangioendolthelioma Dehner Typ I.

In typical hypervascularisation of the focuses, the typical age group (<6 months) and the typical tumor markers (AFP, ß-HCG, catecholamine metaboltites) allows the sonographic/doppler sonographic diagnosis to be made.
In vague constellation, in particular a non-hypervascularized tumor (because of hemorrhage) , an open biopsy should be done.



Supplement from 06/18/2004 (F. Schneble):

German:
Bei dem Patienten wurde unsere Verdachtsdiagnose "infantiles Leberhämangioenditheliom" histologisch gesichert. Initial bestand eine deutliche Hepatomegalie bei sonst unauffälliger Klinik. Wir beobachteten daher den klinischen Verlauf und führten regelmäßige Laborkontrollen durch, die unauffällige Leberfunktionsparameter und normale Transaminasen zeigte. Eine Therapie erfolgte nicht. Darunter bildete sich die Lebervergrößerung zurück, in regelmäßigen sonographischen Kontrollen wurden die vorbeschriebenen multiplen Leberhämangiome immer kleiner, die meisten Herde bildeten sich komplett zurück.
Bei der letzten Kontrolle diese Woche (der klinisch völlig symptomfreie Patient ist zwischenzeitlich 22 Monate alt) waren nur noch 3 max. 9 mm durchmessende rund abgegrenzte hypoechogene Herde nachzuweisen.
zwei Sonodokumentationen habe ich angefügt.

Ultrasound 3: Sonographically only 3 hypoechoic structures with max. 9 mm diameter shown.

Ultrasound 4: Within these structures no increased perfusion is shown.  

N/A  

1. Medline:
Kardorff R, Fuchs J, Peuster M, Rodeck B.
Infantile hemangioendothelioma of the liver--sonographic diagnosis and
follow-up
Utraschall Med 2001 Dec;22(6):258-64

2. Medline:
Daller JA,Bueno J, Gutierrez J, Dvorchik I, Towbin RB, Dickman PS.
Hepatic hemangioendothelioma: clinical experience and management strategy
J Pediatr Surg 1999; 34; 98-105  

Infantile hemangioendothelioma of the liver, hemangioendotheliomatosis, multiple liver tumors, hepatomegaly, benign tumor, liver tumor, benign liver tumor, child, childhood, pediatric radiology  

Fritz Schneble, M Ebert. Infantile hemangioendothelioma of the liver. PedRad [serial online] vol 3, no. 2.
URL: www.PedRad.info/?search=20030227121025