Long-term survival and remarkably durable treatment response in a case of inoperable fibrolamellar carcinoma of the liver — a comprehensive analysis of an 8-year chemotherapeutic journey

Abstract

Hepatocellular carcinoma is the most common primary malignancy of the liver. The fibrolamellar subtype of hepatocellular carcinoma (FLHCC) is a rare liver malignancy constituting ~0.5% of liver cancers. FLHCC can be distinguished by its lack of specific risk factors and unique molecular profile. This case study aims to present an 8-year medical history of a 32-year-old patient with unresectable FLHCC and his outstanding response to systemic therapy. The patient was referred to the liver surgery center due to suspicion of liver cancer based on radiological findings. The tumor turned out to be inoperable and a surgical biopsy was performed. Histopathology confirmed fibrolamellar carcinoma. Six transarterial chemoembolization procedures with doxorubicin-eluting microspheres were performed, which resulted in disease control. Subsequently, from April 2013 to December 2021, the patient received 11 lines of systemic treatment. Sorafenib resulted in disease control lasting 28 months. Cisplatin did not trigger any response. Systemic doxorubicin provided 6 months of stabilization. The patient did not respond to subsequent lines of capecitabin plus temozolomide, vinorelbine, cyclophosphamide, or 5-fluorouracil plus interferon α2b. The reintroduction of sorafenib resulted in 7 months of disease stabilization, and subsequent regorafenib, used as the 9th line of treatment, led to an objective response lasting 26 months. Later, two more lines of treatment consisting of cabozantinib and paclitaxel were administered and they were not effective. The patient died in December 2021 due to liver failure and hemorrhage. 

FLHCC treatment needs an individual approach. One can hope that in the future, more data on FLHCC management will be available.