Russian Journal of OncologyRussian Journal of Oncology1028-99842412-9119Eco-Vector9717310.17816/1028-9984-2021-26-1-13-22Research ArticleClinical prognostic factors for fibrolamellar liver carcinomaAntonovaElena Yu.<p>PhD student of the Department of Medicinal Treatment (Chemotherapy № 17 Department)</p>elenaantonova5@mail.ruhttps://orcid.org/0000-0002-9740-3839MorozEkaterina A<p>MD, Cand. Sci. (Med.)</p>moroz-kate@yandex.ruhttps://orcid.org/0000-0002-6775-3678PodlyznyDaniil V.<p>MD, Cand. Sci. (Med.)</p>dr.podluzhny@mail.ruhttps://orcid.org/0000-0001-7375-3378KudashkinNikolai E.<p>MD, Cand. Sci. (Med.)</p>dr.kudashkin@mail.ruhttps://orcid.org/0000-0003-0504-585XVolkovAleksandr Yu.<p>MD, Cand. Sci. (Med.)</p>79164577128@yandex.ruhttps://orcid.org/0000-0003-4412-2256DzhanyanIrina A.<p><span dir="ltr" role="presentation">Surgeon, Department of Medicinal Treatment (Chemotherapy No.17 Department)</span></p>i-dzhanyan@mail.ruhttps://orcid.org/0000-0002-6323-511XLaktionovKonstantin K.<p>MD, Dr. Sci. (Med.), Prof.</p>lkoskos@mail.ruhttps://orcid.org/0000-0003-4469-502XBrederValery V.<p>MD, Dr. Sci. (Med.)</p>vbreder@yandex.ruhttps://orcid.org/0000-0002-6244-4294Blokhin National Medical Research Center of OncologyPletnev City Clinical HospitalPirogov Russian National Research Medical University1501202126113222301202224022022Copyright © 2021, Eco-Vector2021<p><strong><em>BACKGROUND:</em></strong> Fibrolamellar liver carcinoma is a rare subtype of liver cancer with an unexplored etiology. There is no information about the choice of classification for fibrolamellar liver carcinoma (FLC) in the world literature, and data on the prognostic significance of clinical aspects in FLC are very contradictory and require further study.</p>
<p><strong><em>AIM:</em></strong> To evaluate the practical significance of staging systems for operable hepatocellular cancer for fibrolamellar carcinoma (TNM/AJCC, BCLC) as a separate option, to study the influence of clinical aspects (disease stage, gender, age, size of tumor) of patients with FlC on the long-term results of their treatment.</p>
<p><strong><em>MATERIALS AND METHODS:</em></strong> The retrospective study included 34 patients with FlC who underwent radical surgical treatment at the first stage at the Blokhin National Research Institute of Oncology of the Ministry of Health of the Russian Federation from 2005 to 2020.</p>
<p><strong><em>RESULTS:</em></strong> When assessing the prevalence of the disease according to the TNM-8/AJCC system, a significant correlation between the FlC stage and the prognosis was revealed. It is proved that the greatest RFS was achieved in the group of stages IIIIIB, while the smallest RFS was achieved in the group with stage IVB (<em>p</em>=0.002; <em>p</em>=0.000). The highest OS was achieved in the group of stages IIIIIB, while the lowest OS was achieved in the group with stage IVB (<em>p</em>=0.050; <em>p</em>=0.000). The OV of patients with FlC was not dependent</p>
<p><strong><em>CONCLUSIONS:</em></strong> The TNM-8/AJCC classification optimally predicts the results of surgical treatment of FlC. The use of the Barcelona staging system for this subtype of liver cancer is impractical. The size of the primary tumor, as part of the FlC staging system, turned out to be an independent prognostic factor. The age of the patient 20 years correlates with the deterioration of OV.</p>fibrolamellar carcinomaliver tumorforecastTNM classificationBCLCflooragetumor sizeфиброламеллярная карциномаопухоль печенипрогнозTNM классификацияBCLCполвозрастразмер опухоли[Edmondson HA. Differential diagnosis of tumors and tumor-like lesions of liver in infancy and childhood. AMA J Dis Child. 1956;91(2):168–186. doi: 10.1001/archpedi.1956.02060020170015][Darcy DG, Malek MM, Kobos R, et al. Prognostic factors in fibrolamellar hepatocellular carcinoma in young people. J Pediatr Surg. 2015;50(1):153–156. doi: 10.1016/j.jpedsurg.2014.10.039][Mavros MN, Mayo SC, Hyder O, Pawlik TM. A systematic review: treatment and prognosis of patients with fibrolamellar hepatocellular carcinoma. J Am Coll Surg. 2012;215(6):820–830. doi: 10.1016/j.jamcollsurg.2012.08.001][Pinna AD, Iwatsuki S, Lee RG, et al. Treatment of fibrolamellar hepatoma with subtotal hepatectomy or transplantation. Hepatology. 1997;26(4):877–883. doi: 10.1002/hep.510260412][El-Serag HB, Davila JA. Is fibrolamellar carcinoma different from hepatocellular carcinoma? A US population-based study. Hepatology. 2004;39(3):798–803. doi: 10.1002/hep.20096][Hemming AW, Langer B, Sheiner P, et al. Aggressive surgical management of fibrolamellar hepatocellular carcinoma. J Gastrointest Surg. 1997;1(4):342–346. doi: 10.1016/s1091-255x(97)80055-8][Smith M, Tomboc PJ, Markovich B. Fibrolamellar Hepatocellular Carcinoma. In: StatPearls. Treasure Island (FL): StatPearls Publishing; 2022.][Stipa F, Yoon SS, Liau KH, et al. Outcome of patients with fibrolamellar hepatocellular carcinoma. Cancer. 2006;106(6):1331–1338. doi: 10.1002/cncr.21703][Ang CS, Kelley RK, Choti MA, et al. Clinicopathologic characteristics and survival outcomes of patients with fibrolamellar carcinoma: data from the fibrolamellar carcinoma consortium. Gastrointest Cancer Res. 2013;6(1):3–9. PMC3597938][Honeyman JN, Simon EP, Robine N, et al. Detection of a recurrent DNAJB1-PRKACA chimeric transcript in fibrolamellar hepatocellular carcinoma. Science. 2014;343(6174):1010–1014. doi: 10.1126/science.1249484][Graham RP, Jin L, Knutson DL, et al. DNAJB1-PRKACA is specific for fibrolamellar carcinoma. Mod Pathol. 2015;28(6):822–829. doi: 10.1038/modpathol.2015.4][Hemming AW, Langer B, Sheiner P, et al. Aggressive surgical management of fibrolamellar hepatocellular carcinoma. J Gastrointest Surg. 1997;1(4):342-346. doi: 10.1016/s1091-255x(97)80055-8][Torbenson M. Fibrolamellar carcinoma: 2012 update. Scientifica (Cairo). 2012;2012:743790. doi: 10.6064/2012/743790][Dinh TA, Vitucci EC, Wauthier E, et al. Comprehensive analysis of The Cancer Genome Atlas reveals a unique gene and non-coding RNA signature of fibrolamellar carcinoma. Sci Rep. 2017;7:44653. doi: 10.1038/srep44653]