Abstract

Primary clear cell carcinoma of the liver (PCCCL) is an uncommon variant of hepatocellular carcinoma, usually occurring in older patients with longstanding cirrhosis. We report a case of a giant PCCCL rupturing in a young woman without cirrhosis. A 29 year-old female who presented with a three month history of abdominal pain, nausea, vomiting, food intolerance, and unintentional weight loss, was found to have gastric varices and a large liver mass measuring 22.8cm × 17.8 cm × 13.8 cm. The tumor was comprised of sheets of clear cells with delicate vesicular architecture, and was positive for Hepar 1, Glypican 3, and CD34 in a diffuse staining pattern and CD10 in a canalicular staining pattern, establishing the diagnosis of PCCCL. The patient died of hemorrhagic shock secondary to tumor rupture within two weeks of diagnosis. Conclusion: Although PCCCL most often occurs in older patients with cirrhosis, this uncommon variant of HCC can arise in younger patients without a history of liver disease. It is important to diagnose PCCCL early due to the potentially favorable prognosis of this specific cancer and in order to prevent catastrophic complications.

Keywords: Clear cell hepatocellular carcinoma; Immunohistochemistry; Histology