Rahul Seth1*, Venu Madhav Konala2 and Sreedhar Adapa3
1Department of Hematology and Oncology, SUNY Upstate Medical University, USA
2Department of Oncology, Ashland Bellefonte Cancer Center, USA
3Department of Nephrology, Kaweah Delta Medical Center, USA
Primary clear cell carcinoma of the liver is an uncommon morphologic variant of Hepatocellular Carcinoma (HCC). Management of advanced clear cell variant of HCC is challenging with no established treatment guidelines. We report a case of advanced clear cell HCC in a 55-year-old male presenting with a large liver mass measuring 22 cm × 12 cm × 15 cm with tumor thrombi extending to Inferior Vena Cava (IVC) and the right atrium with acute tumor bleeding causing hemoperitoneum, required emergent embolization of hepatic artery. Immunostains show the tumor to be positive for HepPar1, focally positive for CEA (Carcino Embryonic Antigen)-p (canalicular pattern), and focally and weakly positive for pan-cytokeratins, establishing the diagnosis of primary hepatocellular clear cell carcinoma. Positron Emission Tomography-Computed Tomography (PET CT) demonstrated the liver mass with tumor thrombi and lung nodules consistent with metastasis. Tumor profiling showed combined positive score 5 for Programmed Death Ligand 1 (PD-L1) on tumor determined by IHC 22C3 PharmDx test. He was started on combination immunotherapy with Nivolumab plus ipilimumab with an ongoing response after completion of four cycles followed by nivolumab every two weeks without any clinical symptoms and radiologically stable disease.
Seth R, Konala VM, Adapa S. Nivolumab Plus Ipilimumab in Primary Clear Cell Hepatocellular Carcinoma (CCHCC): A Case Report and Literature Review. Ann Clin Case Rep. 2019; 4: 1752..