A 36-year-old man with a 20-year history of nephrotic syndrome with normal renal function and fluctuating urine protein levels had been treated with glucocorticoids and traditional Chinese medicine for more than 10 years. He was first hospitalized due to abnormal liver function. Routine laboratory tests revealed mild abnormal liver function [total bilirubin (TBIL) 41.9 micromol/L, direct bilirubin (DBIL) 22.7 micromol/L, alanine aminotransferase (ALT) 21.6 U/L, aspartate aminotransferase (AST) 81.1 U/L, gamma-glutamyl transferase (GGT) 517.5 U/L, alkaline phosphatase (ALP) 323 U/L]. Doppler ultrasound, CT scan, and contrast-enhanced MRI scan of the upper abdomen demonstrated multiple small masses in the liver, so hemangioma was considered. Also, the enlarged spleen and collateral vessels indicated the presence of portal hypertension. The patient underwent a percutaneous liver biopsy and soon after felt pain and discomfort in the right upper abdomen with transient blood pressure decline. The ultrasound confirmed bleeding and hemorrhagic shock due to the liver biopsy. The patient recovered after fluid rehydration, blood transfusion, and other supportive treatments. Pathological examination of the liver biopsy ( Figure 1A ) revealed congested hepatic sinusoids and peri-sinusoid fibrosis, as well as dilation of the interlobular veins and some extended into the surrounding hepatic sinus. These changes in liver histology were consistent with NCPH features that represent venous outflow stenosis with portal hypertension. There was no evidence of extra-hepatic portal vein obstruction, and the patient was diagnosed with a drug-induced liver injury [the Roussel Uclaf Causality Assessment Method (RUCAM) score 5] and secondary NCPH. Ursodeoxycholic acid was given, and regular outpatient follow-up was performed.
Three months after discharge, an outpatient contrast-enhanced MRI scan of the upper abdomen depicted enlarged hepatic masses. Considering the possibility of malignancy ( Figure 1D ), the patient was readmitted to the hospital. Routine laboratory tests revealed abnormal liver function [albumin (ALB) 21 g/L, TBIL 35.7 micromol/L, DBIL 14.0 micromol/L, ALT 67 U/L, AST 70 U/L, GGT 147 U/L, ALP 240 U/L]. Liver contrast-enhanced ultrasound and positron emission tomography-computed tomography (PET-CT) indicated the possibility of primary liver malignancy with intratumoral hemorrhage. Whole abdominal CTV demonstrated multiple masses in the liver and an enlarged spleen with a spleen-renal venous shunt ( Figure 1E ). The patient was transferred to the hepatobiliary surgery department and underwent laparoscopic resection of the liver lesions. Liver lesion biopsy pathology ( Figures 1B, C ) demonstrated that the tumor cells were slit and cable-like with a vascular network and growth along the liver sinusoids. Immunohistochemistry revealed high expression of CD34, CD31, and ERG (ETS transcription factor).
The patient was diagnosed with primary hepatic angiosarcoma and was treated with lenvatinib. In May 2020, he stopped taking lenvatinib due to progressively exacerbating liver function and died of severe intra-abdominal infection and acute kidney failure.