Clinicopathological features and outcomes of fibrolamellar hepatocellular carcinoma

Sakti Chakrabarti, Sri Harsha Tella, Anuhya Kommalapati, Brandon M. Huffman, Siddhartha Yadav, Irbaz Bin Riaz, Gaurav Goyal, Kabir Mody, Mitesh J Borad, Sean Cleary, Rory Smoot, Amit Mahipal

Research output: Contribution to journalArticle

Abstract

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.

Original languageEnglish (US)
Pages (from-to)554-561
Number of pages8
JournalJournal of Gastrointestinal Oncology
Volume10
Issue number3
DOIs
StatePublished - Jan 1 2019

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Survival
Recurrence
Fibrolamellar hepatocellular carcinoma
Cell Death
Phosphotransferases
Therapeutics
Ligands
Neoplasms
sorafenib

Keywords

  • Fibrolamellar carcinoma
  • Gemcitabine
  • Nivolumab
  • Sorafenib

ASJC Scopus subject areas

  • Oncology
  • Gastroenterology

Cite this

Clinicopathological features and outcomes of fibrolamellar hepatocellular carcinoma. / Chakrabarti, Sakti; Tella, Sri Harsha; Kommalapati, Anuhya; Huffman, Brandon M.; Yadav, Siddhartha; Riaz, Irbaz Bin; Goyal, Gaurav; Mody, Kabir; Borad, Mitesh J; Cleary, Sean; Smoot, Rory; Mahipal, Amit.

In: Journal of Gastrointestinal Oncology, Vol. 10, No. 3, 01.01.2019, p. 554-561.

Research output: Contribution to journalArticle

Chakrabarti, S, Tella, SH, Kommalapati, A, Huffman, BM, Yadav, S, Riaz, IB, Goyal, G, Mody, K, Borad, MJ, Cleary, S, Smoot, R & Mahipal, A 2019, 'Clinicopathological features and outcomes of fibrolamellar hepatocellular carcinoma', Journal of Gastrointestinal Oncology, vol. 10, no. 3, pp. 554-561. https://doi.org/10.21037/jgo.2019.01.35
Chakrabarti S, Tella SH, Kommalapati A, Huffman BM, Yadav S, Riaz IB et al. Clinicopathological features and outcomes of fibrolamellar hepatocellular carcinoma. Journal of Gastrointestinal Oncology. 2019 Jan 1;10(3):554-561. https://doi.org/10.21037/jgo.2019.01.35
Chakrabarti, Sakti ; Tella, Sri Harsha ; Kommalapati, Anuhya ; Huffman, Brandon M. ; Yadav, Siddhartha ; Riaz, Irbaz Bin ; Goyal, Gaurav ; Mody, Kabir ; Borad, Mitesh J ; Cleary, Sean ; Smoot, Rory ; Mahipal, Amit. / Clinicopathological features and outcomes of fibrolamellar hepatocellular carcinoma. In: Journal of Gastrointestinal Oncology. 2019 ; Vol. 10, No. 3. pp. 554-561.
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abstract = "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.",
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AU - Tella, Sri Harsha

AU - Kommalapati, Anuhya

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AU - Yadav, Siddhartha

AU - Riaz, Irbaz Bin

AU - Goyal, Gaurav

AU - Mody, Kabir

AU - Borad, Mitesh J

AU - Cleary, Sean

AU - Smoot, Rory

AU - Mahipal, Amit

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N2 - 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.

AB - 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.

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