Metastatic nonresectable fibrolamellar hepatoma - Prognostic features and natural history

Fibrolamellar hepatoma has a clinical course distinct from that of typical histologic hepatocellular carcinoma. The clinical behavior and prognostic f eatures of nonresectable metastatic fibrolamellar hepatoma have not previou sly been fully addressed and are the focus of this report. Retrospective ch art review of all patients (n = 17) with nonresectable metastatic fibrolame llar hepatoma referred to the Johns Hopkins Oncology Center from 1985 throu gh 1990 was carried out. All patients had hepatic parenchymal involvement a nd regional node metastases at the time of referral. Metastases were limite d to regional nodes in four patients. The remaining patients had lung metas tases (n = it), peritoneal metastases (n = 5), or both (n = 4). To assess t he impact of the fibrolamellar variant, characteristic-matched control pati ents with typical histologic hepatocellular carcinoma were obtained from th e Radiation Therapy Oncology Group database. Actuarial median survival from treatment was 14 months in the patients with fibrolamellar hepatoma and 7. 7 months in the patients with hepatocellular carcinoma (p < 0.001). Karnofs ky performance status and hepatic tumor volume at time of referral were imp ortant prognostic features. Multimodality treatment included radiation ther apy and radiolabelled antibody, cisplatin-based chemotherapy, or both; resu lts are discussed. Thirteen patients died, nine of liver Failure, three of metastatic disease, and one of sepsis. Fibrolamellar histologic type. liver function tests, tumor volume, and patient performance status were signific ant predictors of survival. The cause of death in fibrolamellar hepatoma di ffers considerably from that observed in typical histologic hepatocellular carcinoma in the United States. The techniques of treatment of this uncommo n disease were modeled after advances in the multimodality treatment of hep atocellular carcinoma and are discussed. Median survival was 14 months in p atients with metastatic nonresectable fibrolamellar hepatoma.