122,550 Registered Members as of 10/24/2014.
Abdomen » Hepatobiliary
Hepatic hemangioendothelioma
« Back to Listing
 
 
Presentation A month old neonate presents with hepatomegaly. She was born at 38 weeks via a normal vaginal delivery. An ultrasound of the liver was performed.
 
 
 
Caption: Sagittal sonogram of the liver.
Description: Sagittal view of the liver demonstrates a large multi-cystic mass occupying most of the right lobe. The liver is enlarged.
 
 
 
Caption: Transverse sonogram of the liver.
Description: Transverse view of the right lobe, revealing a multi-cystic mass.
 
 
 
Caption: Transverse view of the right lobe of liver.
Description: Transverse view of the right lobe of liver, demonstrating the anterior branch of the right portal vein entering the mass. The echogenic tissue appears to be the compressed liver parenchyma.
 
 
 
Caption: Transverse superior view of the liver.
Description: Transverse superior image of the liver shows the right hepatic vein, which is draining from the mass.
 
 
 
Caption: Color Doppler study of the mass.
Description: The cystic spaces completely fill with color demonstrating the mass to be very vascular in nature.
 
Differential Diagnosis Hemangio-endothelioma, hepatoblastoma, hamartoma [the latter two are less likely as the mass is completely vascular].

 
Final Diagnosis Hepatic hemangioendothelioma.
 
Discussion Hemangioendotheliomas are benign vascular mesenchymal tumors that present in infancy [usually before 6 months] and may pose problems due to ensuing complications. The liver is the organ most commonly involved; however splenic, tracheal, pulmonary, GI tract, thymic, pancreatic and meningeal involvement have been reported. These patients may also present with cutaneous hemangiomas. Histologically these tumors are anastomosing arterio-venous channels lined by endothelial cells.

The neonate may present with asymptomatic hepatomegaly or with complications such as high output cardiac failure, consumptive coagulopathy with thrombocytopenia [Kasabach - Merritt syndrome], disseminated intravascular coagulation, anemia, and encroachment of the surrounding tissues. These tumors, if small, usually involute spontaneously; however patients may succumb to complications. Extremely rare cases of malignant conversion to angiosarcoma have also been reported.

Ultrasound is the initial imaging modality almost always employed to diagnose this infantile hepatic tumor. Hemangioendotheliomas have a varied appearance ranging from a solitary solid-appearing lesion to a multifocal variegated cystic mass. Ultrasound can accurately arrive at a diagnosis if the vascular cystic component is the predominant feature. High intratumoral flow may be apparent on Doppler. The cystic spaces may be vascular and may completely fill with color on Doppler examination or may represent areas of cystic necrosis. Additional imaging may be required to confirm the diagnosis and is commonly performed with CT to fully determine the extent of the lesion. Sometimes calcifications may be seen in the mass. If the hepatic tumor is large enough, a prenatal diagnosis can also be made, with the tumor appearing as a highly echogenic lesion in the liver.

CT scan appearance has been described the tumor appearing as a hypo- isodense mass, showing significant post contrast enhancement. MRI and nuclear scans can also be performed. Since most of these patients are managed on a medical basis [treated with steroids], follow up volume imaging of these tumors and its perfusion will be necessary, underscoring the importance of careful pretreatment measurements.

Hepatic mesenchymal hamartoma forms the closest differential for this cystic mass. It is important to differentiate it from a hemangioendothelioma, as the former warrants surgery for definitive treatment. On sonography, a hamartoma may appear as a non-specific multicystic mass, with the cysts filled with clear or gelatinous fluid. But a highly vascular mass favors the possibility of a hemangioendothelioma. Sometimes though, only histology can distinguish the two entities.

 
Case References 1.Chen CC, et al. Hepatic hemangioendothelioma in children: analysis of thirteen cases. Acta Paed Taiwan. 2003 Jan-Feb; 44(1): 8-13.

2.Wong DC, Masel JP. Infantile hepatic haemangioendothelioma. Australas Radiol. 1995 May; 39(2): 140-4.

3.Keslar PJ, et al. From the archives of the AFIP. Infantile hemangioendothelioma of the liver revisited. Radiographics. 1993 May; 13(3): 657-70.

4.Berger TM, et al. Imaging diagnosis and follow-up of infantile hepatic haemangioendothelioma: a case report. Eur J Pediatr. 1994 Feb; 153(2): 100-2.

5.Kardorff R, et al. Infantile hemangioendothelioma of the liver--sonographic diagnosis and follow-up. Ultraschall Med. 2001 Dec; 22(6): 258-64.

6.Srivastava DN, et al. Colour Doppler flow imaging of focal hepatic lesions. Australas Radiol. 2000 Aug; 44(3): 285-9.

7.Stocker JT, Ishak KG. Mesenchymal hamartoma of the liver: report of 30 cases and review of the literature. Pediatr Pathol. 1983 Jul-Sep; 1(3): 245-67.

 
Follow Up The neonate was started on steroids, and a year later, the mass appears to have shown a decrease in size on ultrasound.
 
Other contents by this Author