Intrahepatic mass-forming cholangiocarcinoma growing in a giant hepatic hemangioma: A case report

Abstract

Rationale: Hepatic hemangioma (HH) is a common benign tumor with a high number of normal or abnormal blood vessels. Intrahepatic cholangiocarcinoma (ICC) is a relatively common malignant primary hepatic carcinoma (10%-15%) with high incidence rate and high fatality, yet low discovery rate in the early stages. Ultrasonography (US), computed tomography, and magnetic resonance imaging (MRI) are frequently used and indispensable imaging techniques for the diagnosis of hepatic lesions. It is possible to differentiate a liver lesion from HH with high accuracy owing to their different patterns and hemodynamic characteristics.

Patient concerns: A 59-year-old Asian woman was referred to hospital for a hepatic mass, which was 9.0 × 6.5 cm in size, The patient was tested positive for hepatitis B antigen but negative for serum alpha-fetoprotein and carbohydrate antigen 199 and had a slightly elevated carcinoembryonic antigen level (3.56 ng/ml).

Diagnosis: Liver US and MRI were performed. Grey-scale US revealed a huge heterogeneous mass on the right lobe with a point and line-like blood flow signal on Doppler US. Dynamic contrast-enhanced MRI showed heterogeneous annular nodular enhancement in the arterial phase. An initial diagnosis of HH was made based on the clinical history and imaging results; however, histopathologic examination of the liver lesions revealed modest to severe atypical hyperplasia of intrahepatic bile duct epithelium, cancerization, and mid to high differentiated mass-forming type cholangiocarcinoma combined with focal organized hemangioma.

Interventions: The intrahepatic mass-forming cholangiocarcinoma (IMCC) lesion was considered a focal organization of hemangioma during operation and was surgically removed. No routine chemotherapy was performed after the operation.

Outcomes: The IMCC recurred 23 months after surgery, with elevated serum CA19-9 and CA125. Liver damage was evident, and the patient developed jaundice. The patient was discharged without active treatment and died in 4 months.

Lessons: Although preoperative imaging of focal hepatic lesions is indispensable, intraoperative frozen section analysis and histopathological examination remain essential for definitive diagnosis. This is particularly important for high-risk patients and those with suspected malignancy.

Figure 1

Conventional ultrasound image of hepatic hemangioma with hypoechoic region (arrow) in the right lobe. Intercostal section view.

Figure 2

Conventional ultrasound image of hepatic hemangioma with hypoechoic region (arrow) in right lobe. Intercostal section view.

Figure 3

The mass manifested as heterogeneous hypointense on T1W1 and heterogeneous hyperintense on T2W2 with a tuberculiform hyperintense region (arrow) on MRI. MRI = magnetic resonance imaging, T1W1 = T1-weighted images, T2W2 = T2-weighted images.

Figure 4

The mass manifested as heterogeneous hypointense on T1W1 and heterogeneous hyperintense on T2W2 with a tuberculiform hyperintense region (arrow) on MRI. MRI = magnetic resonance imaging, T1W1 = T1-weighted images, T2W2 = T2-weighted images.

Figure 5

The mass manifested as peripheral, discontinuous, nodular hyperenhancement in the arterial phase with a hyper-enhanced area (arrow) on MRI. MRI = magnetic resonance imaging.

Figure 6

The mass manifested as uneven filling of contrast agent at the venous phase with hyperenhanced region at same position in Figure 3 (arrow) on contrast-enhanced MRI. MRI = magnetic resonance imaging.

Figure 7

Hematoxylin and eosin staining of liver lesion tissue; hepatic hemangioma.

Figure 8

Hematoxylin and eosin staining of liver lesion tissue; IMCC area. IMCC = intrahepatic mass-forming cholangiocarcinoma.

Figure 9

Immunohistochemical staining of ICC. Immunohistochemistry revealed positive labeling of cytokeratin 7.

Figure 10

Immunohistochemical staining of ICC. Immunohistochemistry revealed positive labeling of cytokeratin 19.

Figure 11

Immunohistochemical staining of ICC. The section was negative for Glypican-3, thus ruling out hepatocellular carcinoma.

Figure 12

Immunohistochemical staining of ICC. The section was negative for Hepa, thus ruling out hepatocellular carcinoma.