Hepatic Malformations/”liver hemangioma”

The term “liver hemangioma” or hepatic hemangioma is confusing terminology the meaning depends on the actual diagnosis.  Hemangioma of the liver in an infant or child is very different from the hemangioma of the liver seen in adults.

Adult Liver Hemangioma:  According to Dr. Steven Fishman, Boston Children’s Hospital and NOVA advisor, “Liver or Hepatic hemangioma in adult is in fact not a hemangioma at all, but rather a venous malformation.  These lesions are the most common tumor of the liver and are most often found in adult females over the age of 40.  They are usually found by a routine ultrasound or CT scan and may present with no symptoms to the patient.  These malformations will bee seen on examination as a well defined red mass and measure from 1-20 cm in area.  Most hepatic malformations are solitary but about 20% present in multiple lesions.  Rarely a patient will present with abdominal pain, nausea, vomiting or other abdominal pain.  Sometimes these patients will have anemia or a low platelet count, this is because the red blood cells and platelets may be trapped and destroyed by the malformation in the liver.  The only significant risk is that of rupture and bleeding which is quite uncommon.  The most likely cause of rupture or bleeding is trauma, (car accidents) or pregnancy.

Adult Liver Hemangioma is benign and often requires no therapy unless they are very large or pregnancy is likely.  If there is a concern for rupture, the lesions can be excised quite safely by an experienced liver surgeon- familiar with hepatic malformations.  More often conservative medical observations is advised with follow- up ultrasound and or CT scans to observe growth.  Other treatment options included embolization and or steroid management.”

 

Reference:

This information is from a description written for NOVA by DR. Steven Fishman, Boston Children’s Hospital, Harvard School of Medicine. It first appeared in an issue of Hemangioma Newsline published by NOVA in 2000 and was updated in 2007.

 

Pediatric Liver Hemangioma: There are different types of liver hemangioma seen in the pediatric population.  Accurate diagnosis is important for treatment.

Confusing Terminology

  • Hemangioma vs Hemangiomaendothelioma and Kasabach Merrit Phenomenon

Infants presenting with liver hemangioma do not have hemangioma-endothelioma therefore Kasabach Merrit Phenomenon does not exist in infants with liver hemangioma since KMP only exists in endothelioma.

Focal Lesions:   This type of liver hemangioma is detected in early infancy or may be seen by ultrasound prenatally.  They are often asymptomatic with no skin lesions noted.  There may be mild anemia or thrombocytopenia, (drop in platelets).    There may be an AV or Venous Shunt.  It is possible for congestive heart failure to exist in a patient with a focal liver hemangioma.  These lesions are essentially Rapidly Involuting Congenital Hemangioma (RICH).  Management of Focal Hemangioma includes observation, medical management and emolization of the shunts.  Embolization of the AV or Venous shunts can improve the symptoms of cardiac failure.  It is not known if medical management with medications improve or shrink the lesion because this type of liver hemangioma often involutes faster then any medication can work.

Multi focal Lesions: This type of liver hemangioma is similar to an Infantile Hemangioma (IH) seen in the skin.  They are most often asymptomatic and are associated with multiple skin hemangioma.  (patients with 4 or more skin hemangioma may also have a multi focal liver hemangioma)  The liver hemangioma is spherical and there may be arterial shunts.  These lesions are found on MRI or CT scans.  Management of Multi focal lesions includes observation, embolization and medical management.  These lesions often respond to treatment with oral steroids.  Sometimes the live lesion causes complications that make it necessary for are more rapid response, in these cases embolization can be used to more rapidly shrink the hemangioma in the liver.  If no complications are seen, these lesions can be allowed to involute and will generally involute like the skin hemangiomas.

 

Diffuse Lesions:  This type of liver hemangioma is associated with hempatomegaly or pronounced liver enlargement.  This characteristic results in a condition known as Abdominal Compartment Syndrome:  Impaired Ventilation, Impaired Venous Return and Renal Vein Compression.  Hypothyroidism is a common complication of this type of liver hemangioma.  Congestive Heart Failure is not seen.  As the lesion grows there are uniform (homogeneous) spherical lesions that replace the liver cells.    Treatment of this type of liver hemangioma must be aggressive.  Aggressive medical management is necessary to slow the proliferation of the hemangioma.  Thyroid replacement therapy must be AGGRESSIVE.

Patients with Diffuse Liver Hemangioma likely have severe hypothyroidism which is caused bye type 3 Iodothyronine deiodinase, this enzyme cleaves off iodine and inactivates it the body.  Aggressive Thyroid Replacement therapy may balance this mechanism.  Monitoring TSH levels can help determine the dosage of thyroid replacement medication to use.  Once the liver Hemangioma has involuted thyroid replacement therapy can be discontinued.   Traditionally the management of this type of liver hemangioma did not include Aggressive Thyroid Replacement Therapy, the hemangioma was managed but the child was impaired mentally due to hypothyroidism.  Understanding the management of TSH levels has prevented this complication. 

References:

Huang SA, tu HM, Harney JW Fishman SJ  New England Journal of Medicine 2000; 343:185-189

Fishman, Steven J Vascular Anomalies 2007: Update on Research & Controversies in Clinical Management, April 27-28, 2007 NYU Medical Center

 

NOVA provides this information as a resource.  It is not intended to engage in the practice of medicine or to replace the physician.  NOVA does not claim to have medical knowledge.  NOVA does not endorse any particular physician, treating facility or treatment protocol.  In all cases NOVA, the NOVA Board of Directors and associates recommends that you seek the opinion of a physician experienced in the diagnosis and treatment of hemangioma and vascular malformations.

rev. 1/12/2009

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