PMC5475262_01
Axial (a) and coronal (b) post-contrast CT, obtained during venous phase, show marked hepatomegaly with numerous hepatic lesions in left lobe and in segment 5. The lesions show peripheral enhancement and diminished central attenuation on venous phase. Note the enlarged hepatic arteries (arrow in b).
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His workup prior to presentation at our institution included an initial abdominal ultrasound that reported multifocal masses within the liver, and subsequent CT scan of the abdomen and pelvis with oral and venous phase IV contrast that showed marked hepatomegaly with liver extension into the pelvis and, numerous bilobar hepatic lesions predominantly in segments IV and V (Fig. 1).
Axial post-contrast T1-weighted images through the segment 4 dominant lesion (arrows), obtained during arterial phase (a) and with 3 min delay (b) show peripheral enhancement with progressive centripetal filling. There is no arterial hyperenhancement or washout. The lesions shows central hyperintensity on T2-weighted image (c) and peripheral diffusion restriction on ADC map (d). Note peripheral reticular areas of T2 hyperintensity and enhancement in the background liver suggestive of sinusoidal dilatation.
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The dominant lesions showed central T2 hyperintensity and peripheral diffusion restriction with progressive centripetal enhancement suggestive of fibrolamellar HCC versus cholangiocarcioma, although the former was favored based on the patient's demographics (Fig. 2).
Macroscopic image of resected liver (38 x 19 x 11 cm; weight: 3.3 kg) with multiple slices through tumor (25 cm in greatest dimension).
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On pathologic examination, the specimen weighed 3.3 kg and measured 38 x 19 x 11 cm with tumor measuring 25 x 12.5 x 11 cm and occupying 75% of the capsular surface (Fig. 3).
a. Two representative sections of the tumor; tumor with trabecular and glandular patterns (blue star) shows focal hepatocellular phenotype (positive Arginase-1, rare positive cells with HepPar-1 and strongly positive cholangiocarcinoma phenotype CK7, CK19, Ca19.9). Adjacent section of tumor shows a solid pattern with neuroendocrine differentiation. b. Section of lymph node with metastatic carcinoma. Immunohistochemical stains show predominant neuroendocrine differentiation (positive synaptophysin and CD56 markers).
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Representative sections of the tumor including sections of the paraaortic lymph node were submitted to extensive workup using mainly hepatocellular (HepPar-1, glutamine synthetase, Arginase-1, CAM5.2, AFP, Glypica-3, Beta catenin), adenocarcinoma (CK7, CK19, Ca19.9, MOC31,CK20), and neuroendocrine markers (synaptophysin, chromogranin, CD56 and Ki67) (Fig. 4).