Indian Journal of Radiology Indian Journal of Radiology  

   Login   | Users online: 678

Home Bookmark this page Print this page Email this page Small font sizeDefault font size Increase font size     

 

ABDOMINAL RADIOLOGY Table of Contents   
Year : 2006  |  Volume : 16  |  Issue : 3  |  Page : 363-365
Uncommon presentation of infantile hepatic hemangioendothilioma


From the Elbit Medical Diagnostic Ltd. Hyderabad, India

Click here for correspondence address and email
 

Keywords: Infantile Hepatic Hemangioendothilioma, multifocal, Benign Hepatic Tumors

How to cite this article:
Rao C J, Reddy S L. Uncommon presentation of infantile hepatic hemangioendothilioma. Indian J Radiol Imaging 2006;16:363-5

How to cite this URL:
Rao C J, Reddy S L. Uncommon presentation of infantile hepatic hemangioendothilioma. Indian J Radiol Imaging [serial online] 2006 [cited 2019 Dec 13];16:363-5. Available from: http://www.ijri.org/text.asp?2006/16/3/363/29018

   Introduction Top


Infantile hemangioendothelioma is a type of capillary hemangioma which is seen almost exclusively in children. It is the most common symptomatic vascular tumor, with 85% of cases presenting in the first 6 months. They present with hepatomegaly or as an abdominal mass. Grossly, the lesions are usually well circumscribed and may be focal, multifocal or diffuse. We report a case of infantile hepatic hemangioendothelioma with an uncommon presentation as multiple lesions. The classic features of this disease like early peripheral enhancement, arteriovenous shunting and narrowing of the aorta below the origin of celiac axis are well appreciated by these imaging modalities.


   Case Report Top


A 3 months old boy presenting with an abdominal mass was referred for a CT examination. An ultrasound was performed before the baby was investigated with CT.

On Ultrasonography, the liver showed hepatomegaly with multiple, well defined, hypoechoic lesions of varying sizes[Figure - 1] . On color Doppler imaging abnormally large vessels with high flow were seen in the liver indicating the arteriovenous shunting[Figure - 2]. NECT showed hepatomegaly with multiple well circumscribed hypodense lesions[Figure - 3]. On contrast administration all the lesions showed early massive enhancement with some showing diffuse and the others peripheral enhancement in the arterial phase with filling up of the center on the delayed scans[Figure - 4]. MRI was performed which showed the lesions to be hypointense on T1 weighted imaging and hyperintense on T2[Figure - 5][Figure - 6] and IR[Figure - 7][Figure - 8][Figure - 9].


   Discussion Top


Benign hepatic tumors account for 1/3rd of all hepatic tumors in children.Infantile hemangioendothelioma is the most common benign hepatic tumor in the first 6 months of life. The modes of presentation in decreasing order of frequency are cutaneous hemangiomas, high output CHF secondary to AV shunts and abdominal mass secondary to hepatomegaly. Girls are more frequently affected than boys. Microscopically they consist of multiple anastomosing thick walled vascular spaces lined by plump endothelial cells[1]. They are classified into Type- I tumors where the vascular channels are lined by a single layer of cytologically bland endothelial cells and Type 2 tumors lined by more pleomorphic and hyperchromatic endothelical cells with intra vascular budding and branching[2].

Infantile hemangioendothelioma presents in two forms - focal and diffuse. Diffuse involvement of the liver is much more common than focal lesions. It can vary in size from several mm to 20 cms and presents either as a solitary mass or multiple masses. There is enlargement of celiac, hepatic arteries and proximal aorta with rapid decrease in aortic caliber below the celiac trunk. Kasabach Merrit Syndrome is characterized by an association of hemangioma or hemangioendothelioma with thrombocytopenia and purpura.

Plain abdominal x-ray shows fine speckled calcification in the right hypochondrium and cardiomegaly being a frequent finding is noted on chest x-ray[3]. On Sonography they are predominantly hypoechoic or mixed echogenic lesions with increased flow in the liver. CDI demonstrates no appreciable flow within the lesions.

CT gives the exact size and spread of the lesions. Pre contrast scans show solitary or multiple, homogenous, circumscribed areas with reduced attenuation values[4]. After injection of a bolus of contrast material the lesion shows early massive enhancement which is either diffuse or peripheral[4].

On MRI they are hypointense on T1 with flow voids representing the dilated vessels. They are extremely bright on the T2 weighted images[5]. On Tc-99m RBC hepatic scintigraphy an early blush is seen which is a diagnostic feature of infantile hemangio endothelioma[6].

On MRI they are hypointense on T1 with flow voids representing the dilated vessels. They are extremely bright on the T2 weighted images[5]. On Tc-99m RBC hepatic scintigraphy an early blush is seen which is a diagnostic feature of infantile hemangio endothelioma[6].

They differ in appearance from cavernous hemangiomas on angiography. It is seen as a hypervascular tumor with enlarged, tortuous feeding arteries and stretched intrahepatic vessels. There is pooling of contrast material in sinusoidal lakes with rapid clearing through early draining veins.

If the patient is asymptomatic no treatment is necessary as it is self involuting.Reduction in the size of the lesions can be achieved with steroids, radiotherapy or chemotherapy. If there is no response with medical treatment then therapeutic embolisation using polyvinyl alcohol can be performed. Usually infantile hemangioendothelioma are said to resolve within 2 years.Surgery is reserved for cases where congestive heart failure or coagulopathy cannot be managed medically.

 
   References Top

1.PJ Keslar, JL Buck and DM Selby. From the archives of the AFIP. Infantile hemangioendothelioma of the liver revisited. RadioGraphics 1993; Vol 13: 657-670.  Back to cited text no. 1    
2.Dehner LP, Ishak KG. Vascular tumors of the liver in infants and children : a study of 30 cases and review of the literature. Arch Pathol 1971; 92:101-11.  Back to cited text no. 2  [PUBMED]  
3.Rajendran V R, Manoj B.Giant Infantile Hemangioendothelioma Liver in a two month old baby with a normal Identical Twin-A Case Report. Calicut Medical Journal 2004;2(1):e6.  Back to cited text no. 3    
4.Lucaya J, Enriquez G, Amat L et al. Computed tomography of infantile hepatic hemangioendothelioma. AJR 1985 April; 144 (4) : 821-6.  Back to cited text no. 4    
5.Presedo A, Martinez-Ibanez V, Castellote A et al. Infantile hepatic hemangioendothelioma of the liver : report of the 11 cases. Cir Pediatr 1996 April ; 9(2) : 51-4.  Back to cited text no. 5    
6.Park CH, Hwang HS, Hong J et al. Giant infantile hemangioendothelioma of the liver. Scintigraphic diagnosis. Clin Nucl Med. 1996 April ; 21 (4) : 293-5.  Back to cited text no. 6    

Top
Correspondence Address:
C J Rao
Elbit Medical Diagnostic Ltd. Plot No 435/A, Road No 20, Jubilee Hills, Hyderabad
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-3026.29018

Rights and Permissions


    Figures

[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6], [Figure - 7], [Figure - 8], [Figure - 9]



 

Top
 
  Search
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Email Alert *
    Add to My List *
* Registration required (free)  


    Introduction
    Case Report
    Discussion
    References
    Article Figures

 Article Access Statistics
    Viewed2660    
    Printed95    
    Emailed0    
    PDF Downloaded235    
    Comments [Add]    

Recommend this journal