Clinical Case Report
Retroperitoneal and intrahepatic metastasis from primary clear cell carcinoma of the liver A case report and review of the literature Junjie Xiong, MDa, Du He, MDb, Weiming Hu, MDa, Xubao Liu, MD, PhDa,
Abstract Background: Hepatocellular carcinoma (HCC) is a major cause of cancer-related mortality worldwide and the incidence is
increasing as a result of growing hepatitis B and C virus infections. Primary clear cell carcinoma of the liver (PCCCL) is a rare subgroup of primary HCC, which has low metastatic potential and infrequently reported in literature. Retroperitoneal and intrahepatic metastasis of PCCCL has not been reported previously. Case Summary: Here, we present a 55-year-old male with retroperitoneal and intrahepatic metastasis of PCCCL who is managed with surgical method and transcatheter arterial chemoembolization (TACE) at our institution. When the patient is followed up in 16 months after surgery and TACE, he is alive without any extrahepatic metastasis and abnomal liver function. Conclusion: We concluded that surgical resection of retroperitoneal metastasis and TACE of the intrahepatic tumors provided an
appropriate strategy for the patient with unresectable PCCCL accompanied with extra-hepatic metastasis. Abbreviations: AFP = alpha-fetoprotein, CA-125 = carbohydrate antigen-125, CA19–9 = carbohydrate antigen 19–9, CEA = carcinoembryonic antigen, CgA = chromogranin A, EMA = epithelial membrane antigen, HBV = hepatitis B virus, HCC = hepatocellular carcinoma, LFTs = liver function tests, MART-1 = melanoma antigen recognized by T cells 1, PCCCL = primary clear cell carcinoma of the liver, RCC = renal cell carcinoma, TACE = transcatheter arterial chemoembolization. Keywords: clear cell carcinoma, hepatocellular carcinoma, intrahepatic metastasis, retroperitoneal metastasis, transcatheter arterial chemoembolization
eosin. Histologically, the tumor cells are moderately differentiated with low metastatic potential and diagnosis is usually made by correlation of histopathology, tumor markers, and immunohistochemistry. Treatment and prognosis of PCCCL with distal organ metastasis is infrequently reported in literature. We present an interesting case of PCCCL associated with retroperitoneal and intrahepatic metastasis in a hepatitis B virus (HBV)-related cirrhotic patient with surgical resection and transcatheter arterial chemoembolization (TACE) management. To the best of our knowledge, this is the ﬁrst report of such a case in the worldwide.
1. Introduction Hepatocellular carcinoma (HCC) is the ﬁfth most common cancer and the third leading cause of cancer-related deaths worldwide. About 80% cases are reported from Asian countries, accounted for by the high prevalence of Hepatitis B and C infections in this region. Extrahepatic metastasis of HCC at the time of initial diagnosis, even if the primary lesion is locally advanced, is however a rare phenomena. Primary clear cell carcinoma of the liver (PCCCL) is a rare type of HCC, pathologically characterized by diffuse clear cells of the tumor, showing a clear cytoplasm that does not stain with hematoxylin-
2. Case presentation Editor: Peng Qi.
This study was approved by the Institutional Ethic Committee of West China Hospital, Sichuan University. Written informed consent was obtained from this patient. The patient was a 55year-old male who presented with a 1-month history of persistent right upper quadrant abdominal pain referred to the right shoulder, associated with episodes of chills and fever along with 5 kg weight loss and anorexia. There were no signiﬁcant medical co-morbidities, other than positive Hep B status, or medication history and there was a 30 pack year history of smoking with moderate alcohol intake. On examination, the patient was hemodynamically stable with no evidence of jaundice and had some tenderness in the right upper quadrant. Investigations revealed a white blood cell count of 11,730/mm3 with necrophilia (87%). Liver function tests (LFTs) were normal other than direct bilirubin, 9.8 mmol/L (normal range,