Hepatic Hemangiomas Clinical Presentation
- Author: David C Wolf, MD, FACP, FACG, AGAF; Chief Editor: Julian Katz, MD more...
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.[9]
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.[10]
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,[11] and (4) fever of unknown origin.[12]
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.[13]
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 [8]
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.[14]
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.[15]
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.
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