eMedicine Specialties > Gastroenterology > Liver

Hemangiomas, Hepatic

Author: David C Wolf, MD, FACP, FACG, AGAF, Medical Director of Liver Transplantation, Westchester Medical Center, Professor of Clinical Medicine, Division of Gastroenterology and Hepatobiliary Diseases, Department of Medicine, New York Medical College
Coauthor(s): Unnithan V Raghuraman, MD, FRCP, FACG, FACP, Consulting Staff, Department of Gastroenterology, St John Medical Center
Contributor Information and Disclosures

Updated: Dec 22, 2008

Introduction

Background

Hemangioma is the most common benign tumor affecting the liver. Hepatic hemangiomas are mesenchymal in origin and usually are solitary. Some authorities consider them to be benign congenital hamartomas. Hemangiomas are composed of masses of blood vessels that are atypical or irregular in arrangement and size. Etiology remains unknown.

Pathophysiology

Although no definite familial or genetic mode of inheritance has been described, Moser et al reported a large family of Italian origin in which 3 female patients in 3 successive generations had large symptomatic hepatic hemangiomas.1 The authors postulated that restriction of the disease to the female sex could be explained by sex-dependent differences in penetrance, the expression of a presumed liver-hemangioma gene, or the production of proliferative factors, such as female sex hormones.

Several pharmacologic agents have been postulated to promote tumor growth. Steroid therapy2 , estrogen therapy, and pregnancy3 can increase the size of an already existing hemangioma. One study prospectively evaluated 94 women with hepatic hemangiomas, with a mean follow-up period of 7.3 years (range, 1-17 y).4 An increase in the size of the hemangiomas was seen in 23% of women who received hormonal therapy as opposed to 10% of control subjects (P=0.05). Hemangiomas have also been reported in pregnant women following ovarian stimulation therapy with clomiphene citrate and human chorionic gonadotropin.5

Frequency

United States

The reported incidence rate of hepatic hemangiomas is approximately 2%. The prevalence rate at necropsy is as high as 7.4%. The widespread use of noninvasive abdominal imaging modalities has led to increased detection of asymptomatic lesions in vivo.

Sex

Women, especially with a history of multiparity, are affected more often than men. The female-to-male ratio is 4-6:1.

Age

Hepatic hemangiomas can occur at all ages. Most hepatic hemangiomas are diagnosed in individuals aged 30-50 years.

  • Female patients often present at a younger age and with larger tumors.
  • Hepatic hemangiomas may be seen in infancy. They have also been detected prenatally in a growing fetus.6

Clinical

History

  • Hemangiomas present a diagnostic challenge because they can be mistaken for hypervascular malignancies of the liver and can coexist with (and occasionally mimic) other benign and malignant hepatic lesions, including focal nodular hyperplasia, hepatic adenoma, hepatic cysts, hemangioendothelioma, hepatic metastasis, and primary hepatocellular carcinoma.
  • Hepatic hemangiomas can occur as part of well-defined clinical syndromes.
    • In Klippel-Trenaunay-Weber syndrome, hepatic hemangiomas occur in association with congenital hemiatrophy and nevus flammeus, with or without hemimeganencephaly.
    • In Kasabach-Merritt syndrome, giant hepatic hemangiomas are associated with thrombocytopenia and intravascular coagulation.
    • Osler-Rendu-Weber disease is characterized by numerous small hemangiomas of the face, nares, lips, tongue, oral mucosa, gastrointestinal tract, and liver.
    • Von Hippel-Lindau disease is marked by cerebellar and retinal angiomas, with lesions also in the liver and pancreas.
    • Multiple hepatic hemangiomas have been reported in patients with systemic lupus erythematosus.7
  • Clinical features
    • Hepatic hemangiomas are more common in the right lobe of the liver than in the left lobe. 
    • Hemangiomas of the liver are usually small and asymptomatic. They are most often discovered when the liver is imaged for another reason or when the liver is examined at laparotomy or autopsy. Larger and multiple lesions may produce symptoms. Goodman noted that symptoms are experienced by 40% of patients with 4-cm hemangiomas and by 90% of patients with 10-cm hemangiomas.8
    • Right upper quadrant pain or fullness is the most common complaint. In some cases, pain is explained by thrombosis and infarction of the lesion, hemorrhage into the lesion, or compression of adjacent tissues or organs. In other cases, pain is unexplained.
    • The only findings upon physical examination are, infrequently, an enlarged liver or the presence of an arterial bruit over the right upper quadrant.
    • Rarely, hemangiomas may present as a large abdominal mass. Other atypical presentations include the following: (1) cardiac failure from massive arteriovenous shunting, (2) jaundice from compression of the bile ducts, (3) gastrointestinal bleeding from hemobilia,9 and (4) fever of unknown origin.10
    • An illness that resembles a systematic inflammatory process has been described with findings of fever, weight loss, anemia, thrombocytosis, increased fibrinogen level, and elevated erythrocyte sedimentation rate.11
  • Complications
    • Complications depend on the size and location of the tumor.
    • Rarely, large tumors rupture spontaneously or after blunt trauma.  Patients may present with signs of circulatory shock and hemoperitoneum.
    • Early satiety, nausea, and vomiting may occur when large lesions compress the stomach, producing gastric outlet obstruction.
    • One case has been reported of lower extremity edema caused by compression of the inferior vena cava by a cavernous hemangioma of the caudate lobe of the liver.
  • Infantile hemangiomas
    • Hepatic hemangiomas may be seen in 5-10% of children aged 1 year. They typically regress during childhood.
    • Reports have described infants with massive hepatic hemangiomas and hypothyroidism. In these cases, the tumor was found to express type 3 iodothyronine deiodinase, which resulted in an increased rate of inactivation of thyroid hormone.12

Physical

Infrequently, patients may present with an enlarged liver, an abdominal mass, or an arterial bruit over the right upper quadrant.   
 
Cutaneous hemangiomas are a common finding. It is unclear whether or not they are associated with hepatic hemangiomas.13

Causes

Oral contraceptives and steroids may accelerate the growth of a hemangioma. Whether or not these drugs actually induce the formation of the hemangioma is unclear.

More on Hemangiomas, Hepatic

Overview: Hemangiomas, Hepatic
Differential Diagnoses & Workup: Hemangiomas, Hepatic
Treatment & Medication: Hemangiomas, Hepatic
Follow-up: Hemangiomas, Hepatic
References
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References

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Further Reading

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Keywords

hepatic hemangiomas, hepatic hemangioma, hemangioma, cavernous hemangioma, liver tumor, liver lesions, liver hemangioma

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Contributor Information and Disclosures

Author

David C Wolf, MD, FACP, FACG, AGAF, Medical Director of Liver Transplantation, Westchester Medical Center, Professor of Clinical Medicine, Division of Gastroenterology and Hepatobiliary Diseases, Department of Medicine, New York Medical College
David C Wolf, MD, FACP, FACG, AGAF is a member of the following medical societies: American Association for the Study of Liver Diseases, American College of Gastroenterology, American College of Physicians, and American Gastroenterological Association
Disclosure: Nothing to disclose.

Coauthor(s)

Unnithan V Raghuraman, MD, FRCP, FACG, FACP, Consulting Staff, Department of Gastroenterology, St John Medical Center
Unnithan V Raghuraman, MD, FRCP, FACG, FACP is a member of the following medical societies: American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, and American Society of Gastrointestinal Endoscopy
Disclosure: Nothing to disclose.

Medical Editor

Vivek V Gumaste, MD, Associate Professor of Medicine, Mt Sinai School of Medicine, Adjunct Clinical Assistant, Mt Sinai Hospital; Director, Division of Gastroenterology, City Hospital Center, Elmhurst, NY
Vivek V Gumaste, MD is a member of the following medical societies: American College of Gastroenterology and American Gastroenterological Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Oscar S Brann, MD, FACP, Associate Clinical Professor, Department of Medicine, University of California at San Diego; Consulting Staff, Mecklenburg Medical Group
Oscar S Brann, MD, FACP is a member of the following medical societies: American Gastroenterological Association
Disclosure: Nothing to disclose.

CME Editor

Alex J Mechaber, MD, FACP, Associate Dean for Undergraduate Medical Education, Associate Professor of Medicine, University of Miami Miller School of Medicine
Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine
Disclosure: Nothing to disclose.

Chief Editor

Julian Katz, MD, Clinical Professor of Medicine, Drexel University College of Medicine; Consulting Staff, Department of Medicine, Section of Gastroenterology and Hepatology, Hospital of the Medical College of Pennsylvania
Julian Katz, MD is a member of the following medical societies: American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Geriatrics Society, American Medical Association, American Society for Gastrointestinal Endoscopy, American Society of Law Medicine and Ethics, American Trauma Society, Association of American Medical Colleges, and Physicians for Social Responsibility
Disclosure: Nothing to disclose.

 
 
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