A liver lesion with systemic inflammatory manifestations.

A 50-year-old Caucasian man presented with recurrent fevers, anorexia, and 15 kg weight loss over 10 weeks. He had no abdominal pain, jaundice, or bleeding in stools. He did not smoke or drink alcohol. On physical examination, his abdomen was soft with an enlarged nontender liver. Laboratory workup showed a white cell count of 6800/mm3, anemia with hemoglobin of 9.4 g/dL, and elevated platelets of 466,000/mm3. Sedimentation rate (ESR) was greater than 140 mm/h and C-reactive protein was 76 mg/dL. Serum iron and transferrin was low with an elevated ferritin consistent with anemia of chronic disease. Liver function tests were normal except for an elevated alkaline phosphatase of 178 U/L. Computed tomography of the abdomen [Figure 1] showed a 20 cm lesion with peripheral and discontinuous nodular enhancement within the majority of the right lobe of the liver. Magnetic resonance imaging [Figure 2] showed a large heterogeneous lesion of 20 cm occupying almost the entire right lobe of the liver. There was hyperintense signal on T2-weighted images with discontinuous centripetal peripheral nodular enhancement. The lesion also had hypointense areas compatible with hemorrhage.

Figure 1

Computed tomography abdomen showing a large 20 cm heterogenous liver lesion, which demonstrates peripheral, nodular centripetal discontinuous delayed enhancement

Figure 2

T2-weighted magnetic resonance imaging shows a large lesion with some hypointense areas compatible with hemorrhage

QUESTION

Q1. What is the diagnosis?

ANSWER

The differential diagnosis of a hypervascular liver lesion includes adenoma, focal nodular hyperplasia, hepatocellular carcinoma, and hypervascular metastases, but based on the imaging findings of peripheral, nodular, centripetal, discontinuous, delayed enhancement, the lesion is consistent with a hemangioma. Hemangiomas are the most common primary tumors of the liver.[1] Because the lesion is greater than 4 cm in size, it is classified as a giant hemangioma. The pathophysiology of inflammatory process in patients with giant hemangioma is not clear. Progressive increase in size may lead to congestion, bleeding, thrombosis, and infarction.[1] Cytokines released by hepatic macrophages and endothelial liver cells, such as IL-1 and IL-6, seem to be the most possible mediators of systemic inflammation.[2] Hemangiomas are usually asymptomatic. Inflammation may result in systemic signs, such as fevers, pain, and weight loss. Laboratory abnormalities reported include anemia, and elevated platelets, fibrinogen, ESR, and C-reactive protein.[3] White blood cell count has been normal in majority of the reported cases. Liver function tests are usually normal, although there may be cholestasis related to biliary compression. Inflammatory signs and symptoms may be mistaken for an infectious process, such as a liver abscess. Kasabach–Merrit syndrome is an uncommon complication, which involves coagulopathy with fibrinolysis and thrombocytopenia associated with a hemangioma.[1] Most patients with giant hemangiomas remain symptom-free and should be clinically observed.[1] When symptoms are present, resection may result in the disappearance of symptoms and laboratory abnormalities.[3]