Hepatic Hemangiomas Clinical Presentation

Updated: Oct 17, 2017
  • Author: David C Wolf, MD, FACP, FACG, AGAF, FAASLD; Chief Editor: BS Anand, MD  more...
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Presentation

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 angiosarcoma, hepatic metastasis, and primary hepatocellular carcinoma. [9]

Hepatic hemangiomas can occur as part of well-defined clinical syndromes. In Klippel-Trenaunay-Weber syndrome, hepatic hemangiomas occur in association with congenital hemi-atrophy and nevus flammeus, with or without hemi-megalencephaly. In Kasabach-Merritt syndrome, giant hepatic hemangiomas are associated with thrombocytopenia and intravascular coagulation. In this condition, patients are typically male and younger than 1 year. [10] The goal of treatment is eradication of the hemangioma, with subsequent control over the patient's coagulopathy. [11]

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. [12]

Infantile hemangioma is a common tumor in infancy. It may be seen in 5-10% of children aged 1 year. The hemangiomas typically regress during childhood. [8] Typically, the tumors affect the skin and the subcutaneous tissue. Occasionally, they affect the liver. Case reports have described the regression of infantile hemangiomas after treatment with propranolol. [13, 14]

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. [15]

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. [9] Larger and multiple lesions may produce symptoms that necessitate surgery. Goodman noted that symptoms are experienced by 40% of patients with 4-cm hemangiomas and by 90% of patients with 10-cm hemangiomas. [16]

Right upper quadrant pain or fullness is the most common complaint. [9] 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, seen infrequently, are an enlarged liver or the presence of an arterial bruit over the right upper quadrant.

Up to 40% of hepatic hemangiomas grow over time, at about a modest rate of 2 mm per year in linear dimension and 17.4% per year in volume, based on data from a 10-year retrospective study. [17] In a separate retrospective study, hemangiomas increased in size in 61.0% of patients, with the highest growth period in those younger than 30 years (0.46 ± 0.41 cm per year) and slowest growth period in those older than 50 years (0.16 ± 0.42 cm per year). [18] Hemangiomas sized 8-10 cm grew at 0.80 ± 0.62 cm per year, whereas those larger than 10 cm grew at a slower rate of 0.47 ± 0.91 cm per year. [18]

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, [19] and (4) fever of unknown origin. [20]

An illness that resembles a systematic inflammatory process has been described with the findings of fever, weight loss, anemia, thrombocytosis, increased fibrinogen level, and elevated erythrocyte sedimentation rate. [21]

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Physical Examination

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 in adults. It is unclear whether or not they are associated with hepatic hemangiomas. [22]

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