Mass-forming intrahepatic cholangiocarcinoma

Case contributed by Dr Bruno Di Muzio

Presentation

Mildly deranged LFTs for investigation.

Patient Data

Age: 65 years
Gender: Female

Liver

Ultrasound

There is a segment II liver lesion relatively well-defined, hypoechoic, and with associated mildly dilated intrahepatic ducts. 

Abdomen and pelvis

CT

An enhancing mass is identified in segment II/IVA measuring 6 x 6 cm with central low attenuation. There is evidence of associated dilated intra-hepatic ducts in segment three.  

There is a thickening of the uterine endometrium for a patient with postmenopausal status, measuring 11 mm.

The remainder of the study is unremarkable. 

The left hepatic lobe is small with atrophic segments II and III and is mostly occupied by a circumscribed mass that shows low T1, mildly elevated T2, and increased DWI signal. Post-contrast, the lesion shows intense hyperenhancement on the arterial phase that gets homogeneous to the background parenchyma on portal venous phase, and starts to wash out on the delayed/intermediate phase, showing mostly low signal compared to the background liver on the hepatobiliary phase. This washout is concerning for a malignant process. Within the lesion, a note is made to dilated intrahepatic ducts, as previously demonstrated on the external scan. No fat demonstrated within the lesion. 

Focal area of arterial hyperenhancement in segment VI without washout. There is no corresponding abnormal DWI signal and homogeneous uptake of hepatobiliary contrast at 20 minutes.  No mass is visible. No other focal liver lesions identified. The appearances are therefore not those of metastasis and most in keeping with a THID/THAD.

The pancreas appears normal. Small left adrenal gland adenoma and normal right adrenal gland. There are features suggestive of splenectomy and a 3.1 cm splenunculus in the splenic bed. Apart from a simple cortical cyst on the left, the imaged kidneys are normal. There is no lymphadenopathy or free fluid within the superior abdomen.

Conclusion: Left hepatic lobe mass has indeterminate imaging features and is concerning for a malignant tumor. Further hepatobiliary discussion with a view for US-guided core-biopsy is recommended.

Case Discussion

The case was further reviewed in an MDM, with the favored diagnosis of cholangiocarcinoma. Further staging has not shown metastatic disease. Surgical resection was offered: 

Macroscopy: Labeled "Left lobe liver".  A segment of the liver with a roughened resection surface 110 x 63 mm (inked black).  Focally, the capsule is retracted over a 120 x 35 mm area (inked green).  There is a small amount of attached fat 50 x 20 mm, within which there is a tubular structure approximately 65 mm long and up to 8mm wide resembling a the cholecystic duct.  This structure corresponds to the amputated staple margin there appears to terminate at the hilum. Mottled irregular grey patches are present over the surface of the capsule overlying the tumor. There is a 30 mm staple line on the resection margin (removed, resulting in surface inked yellow).  Sectioning reveals an irregularly shaped white, firm tumor 70 x 55 x 54 mm extending through the parenchyma and abutting the resection margin and the stapled duct margin.  The background liver is tan-brown to red. 

Microscopy: Sections were taken through the liver, focally showing expanded bile duct within which there is a proliferation of glandular spaces of varying size lined by moderately pleomorphic epithelium. Adjacent to this, there is the extensive infiltration of the adjacent parenchyma by glands of varying caliber lined by moderately pleomorphic epithelium showing nuclei with a slightly vesicular nucleus and small nucleoli and mitoses. In areas, the tumor has a hyalinised connective tissue associated with the infiltrating cells. There is an evident perineural invasion. The tumor is seen to focally extend to the ductal/hilar are margin. Vascular invasion is not seen. Away from this area, the hepatic parenchyma shows portal tract chronic inflammation and evidence of biliary stasis. Bile ducts away from the lesion, show no periductal sclerosis or inflammation extending into the epithelium.
Tumor cells are immunoreactive with cytokeratin 7 and show some intermixed immunoreactivity with cytokeratin 20. There is no reaction with Hepatocyte specific antigen or CD10.

Conclusion: Left lobe of liver -  intrahepatic cholangiocarcinoma showing perineural invasion, present at the ductal/hilar resection margin. Adjacent liver shows no evidence of cholangitis.

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