Which clinical history findings are characteristic of hepatic hemangiomas?

Updated: Jun 17, 2019
  • Author: David C Wolf, MD, FACP, FACG, AGAF, FAASLD; Chief Editor: BS Anand, MD  more...
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Answer

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 a 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, 15, 16]

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 the thyroid hormone. [17]


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