Clinicopathological features and outcomes of fibrolamellar hepatocellular carcinoma

被引:34
|
作者
Chakrabarti, Sakti [1 ]
Tella, Sri Harsha [2 ]
Kommalapati, Anuhya [2 ]
Huffman, Brandon M. [1 ]
Yadav, Siddhartha [1 ]
Bin Riaz, Irbaz [1 ]
Goyal, Gaurav [1 ]
Mody, Kabir [3 ]
Borad, Mitesh [4 ]
Cleary, Sean [5 ]
Smoot, Rory L. [5 ]
Mahipal, Amit [1 ]
机构
[1] Mayo Clin, Dept Med Oncol, Rochester, MN 55905 USA
[2] Univ South Carolina, Sch Med, Dept Internal Med, Columbia, SC 29208 USA
[3] Mayo Clin, Dept Oncol, Jacksonville, FL 32224 USA
[4] Mayo Clin, Dept Oncol, Scottsdale, AZ USA
[5] Mayo Clin, Dept Surg, Rochester, MN 55905 USA
关键词
Fibrolamellar carcinoma; sorafenib; nivolumab; gemcitabine; NATURAL-HISTORY; PROGNOSIS; EPIDEMIOLOGY; SURVIVAL;
D O I
10.21037/jgo.2019.01.35
中图分类号
R73 [肿瘤学];
学科分类号
100214 ;
摘要
Background: Clinicopathological features and the outcomes of patients with fibrolamellar hepatocellular carcinoma (FLHCC) are not clearly defined. Methods: Data were collected by retrospective chart review on 42 patients with FLHCC treated between 1990 and 2017 at Mayo Clinic. Results: Of 42 patients (median age at diagnosis 22 years), 10 patients (23.8%) had stage I disease and 32 patients (76.2%) had stage II to IVB disease. All 10 patients with stage I disease and 21 of 32 patients with stage II-IVB disease underwent resection at presentation. In stage I patient group, 6 patients experienced recurrence with a median time to recurrence of 30.5 months and a 5-year overall survival (OS) of 86%. Patients with stage II to IVB disease who underwent resection (n= 21) upfront had a median OS of 32.5 months and 5-year OS of 44%. In the upfront surgery group, 71% of patients experienced recurrence. The median OS of patients with unresectable disease (n= 11) was 10 months. Four out of nine patients treated with sorafenib had stable disease and one patient with programmed cell death ligand-1 (PD-L1) expressing tumor had a near complete response after 2 months of therapy with nivolumab. Conclusions: In FLHCC, surgical resection was associated with prolonged OS; although most patients had a disease recurrence regardless of disease stage and resection margin status. The response to kinase inhibitor, sorafenib, was variable. In select cases, therapy with a checkpoint inhibitor may provide a viable treatment option.
引用
收藏
页码:554 / 561
页数:8
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