Clinical features and surgical outcomes of fibrolamellar hepatocellular carcinoma: retrospective analysis of a single-center experience

• Published in: World journal of surgical oncology Vol. 18; no. 1; p. 93
• Main Authors: Lemekhova, Anastasia, Hornuss, Daniel, Polychronidis, Georgios, Mayer, Philipp, Rupp, Christian, Longerich, Thomas, Weiss, Karl-Heinz, Büchler, Markus, Mehrabi, Arianeb, Hoffmann, Katrin
• Format: Journal Article
• Language: English
• Published: England BioMed Central Ltd 12.05.2020; BioMed Central; BMC
• Subjects: Abdomen; Adult; Biopsy; Cancer therapies; Carcinoma, Hepatocellular - complications; Carcinoma, Hepatocellular - diagnosis; Carcinoma, Hepatocellular - mortality; Carcinoma, Hepatocellular - surgery; Care and treatment; Cirrhosis; Contrast Media - administration & dosage; Diagnosis, Differential; Differential diagnosis; Disease; Disease-Free Survival; Female; Fibrolamellar hepatocellular carcinoma; FL-HCC; Follow-Up Studies; Health aspects; Health services; Hepatectomy; Hepatocellular carcinoma; Hospitals; Human; Humans; Incidental Findings; Laboratories; Liver; Liver - diagnostic imaging; Liver - pathology; Liver - surgery; Liver cancer; Liver cirrhosis; Liver diseases; Liver Neoplasms - diagnosis; Liver Neoplasms - mortality; Liver Neoplasms - pathology; Liver Neoplasms - surgery; Lymph nodes; Lymphatic Metastasis - therapy; Magnetic Resonance Imaging; Male; Medical prognosis; Metastases; Metastasis; Morbidity; Mortality; Neoplasm Staging; Paraneoplastic; Paraneoplastic Syndromes - diagnosis; Paraneoplastic Syndromes - etiology; Patient outcomes; Patients; Physiological aspects; Prognosis; Retrospective Studies; Signs and symptoms; Surgery; Surgical outcome; Surgical outcomes; Survival; Thromboembolism; Thromboembolism - diagnosis; Thromboembolism - etiology; Tomography, X-Ray Computed; Tumors; Young Adult; Young adults; Germany; Human; Thrombocytopenia; Paraneoplastic; Fibrolamellar hepatocellular carcinoma; FL-HCC; Hepatocellular carcinoma; Thromboembolism; Surgical outcome
• ISSN: 1477-7819; 1477-7819
• DOI: 10.1186/s12957-020-01855-2

Clinicopathological features and surgical outcomes of patients with fibrolamellar hepatocellular carcinoma (FL-HCC) are underreported. The aim of this study is to describe clinical characteristics and surgical outcomes for patients with this rare tumor to raise awareness among clinicians and surgeons. Retrospective review of records of a tertiary referral center and specialized liver unit was performed. Out of 3623 patients who underwent liver resection, 366 patients received surgical treatment for HCC; of them, eight (2.2%) had FL-HCC and were resected between October 2001 and December 2018. Eight patients (3 males and 5 females) with FL-HCC (median age 26 years) underwent primary surgical treatment. All patients presented with unspecific symptoms or were diagnosed as incidental finding. No patient had cirrhosis or other underlying liver diseases. Coincidentally, three patients (37.5%) had a thromboembolic event prior to admission. The majority of patients had BCLC stage C and UICC stage IIIB/IVA; four patients (50%) presented with lymph node metastases. The median follow-up period was 33.5 months. The 1-year survival was 71.4%, and 3-year survival was 57.1%. Median survival was at 36.4 months. Five patients (62.5%) developed recurrent disease after a median disease-free survival of 9 months. Two patients (25.0%) received re-resection. FL-HCC is a rare differential diagnosis of liver masses in young patients. Since the prognosis is limited, patients with incidental liver tumors or lesions with suspicious features in an otherwise healthy liver should be presented at a specialized hepatobiliary unit. Thromboembolism might be an early paraneoplastic symptom and needs to be elucidated further in the context of FL-HCC.