Infantile hepatic haemangioma

Infantile hepatic haemangiomas (IHH) are a liver lesion composed of large endothelial-lined vascular channels seen in fetuses and neonates. It should not be confused with a hepatic epithelioid haemangioendothelioma, which occurs in older patients.

Those benign tumours were previously referred as hepatic infantile haemangioendotheliomas, but as they are similar to haemangiomas elsewhere in the body, they have been reclassified as haemangiomas by the International Society for the Study of Vascular Anomalies (ISSVA). 

IHH occur in fetuses and neonates, and have been detected in utero as early as at 16 weeks of gestation. It is the most frequent liver mass in infants (<6 months).

IHH have substantial arteriovenous shunting which may lead to fetal cardiovascular compromise and hydrops fetalis. It may present as hepatomegaly since the entire liver is involved in most cases.

In addition, fetuses may also develop haemolytic anaemia, thrombocytopenia, and consumptive coagulopathy (Kasabach-Merritt sequence).

If these tumours are not detected prenatally, neonates may present with unexplained congestive heart failure.

IHH are histologically similar to haemangiomas (strawberry nevus) but distinct from both adult type cavernous haemangiomas which are venous malformations and angiosarcomas.

Markers

In 3% of cases there is elevated AFP in serum 6.

Associations
  • hypothyroidism: probably due to high levels of type 3 iodothyronine deiodinase activity produced by haemangiomas 3
  • haemangiomata elsewhere in 10% of cases
Plain radiograph

Nonspecific features such as hepatomegaly and liver calcifications, which is present in ~15% of the cases, may be seen. Indirect signs of congestive heart failure may be present on a chest radiograph. 

Ultrasound

Infantile hepatic haemangiomas have a variable sonographic appearance and may be either hypoechoic or hyperechoic or may have mixed echogenicity with prominent vascular channels. Colour Doppler sonographic evaluation will show increased flow.

CT

There is typical peripheral enhancement with gradual filling in. Another characteristic finding is reduction in the aortic caliber (mid-aortic syndrome) below the level of coeliac branch because of the important vascular distribution toward the liver. The same process will cause coeliac trunk and hepatic artery hypertrophy.

MRI

Multifocal haemangiomas are spherical lesions with homogeneous signal intensity on MRI. Large flow voids are usually present. Typical signal characteristics include:

  • T1: hypointense 3
  • T2: hyperintense 3

They generally demonstrate uniform enhancement and may demonstrate the same vascular changes as seen on CT.

The natural history of IHH in infancy is a rapid, proliferative growth phase in the first six months of life, followed by regression and involution.

If the child remains asymptomatic, no treatment may be needed.

If symptoms of high output cardiac failure occur, the first line of therapy is propranolol. If medical treatment fails then, the lesions may be embolised to control any AV shunting causing the cardiac failure.

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Article information

rID: 12168
Section: Pathology
Tag: cases
Synonyms or Alternate Spellings:
  • Infantile hemangioendothelioma
  • Hepatic hemangioendothelioma
  • Hepatic haemangioendothelioma
  • Hepatic infantile haemangioendothelioma
  • Infantile hemangioendothelioma of the liver
  • Infantile haemangioendothelioma of liver
  • Infantile hepatic haemangioendothelioma
  • Infantile hepatic hemangiomas
  • Infantile hepatic hemangioma
  • Hepatic infantile hemangioendothelioma

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