Hepatocarcinoma fibrolamelar
Presentación de un caso clínico
Palabras clave:
CARCINOMA HEPATOCELULAR, FIBROLAMELLAR HEPATOCARCINOMA
Resumen
El hepatocarcinoma fibrolamelar es una entidad poco frecuente cuya incidencia varía entre 1% y 5% en el porcentaje de todos los hepatocarcinomas. Afecta principalmente a pacientes jóvenes con hígado sano, y en el 50% de los casos su diagnóstico se realiza en etapas avanzadas de la enfermedad. Se presenta el caso de un paciente de 15 años de edad, sano, que consulta por tumoración abdominal de tres meses de evolución, dolor en epigastrio y adelgazamiento. Los estudios de imagen informan tumoración que sustituye el lóbulo izquierdo del hígado con realce heterogéneo en la fase arterial, que infiltra la vena suprahepática izquierda. Además, informa lesiones en los segmentos V y VIII, extensas adenopatías en el hilio hepático, y nódulos a nivel peritoneal y subpleural. Se realizó punción biópsica hepática que confirmó el diagnóstico de hepatocarcinoma fibrolamelar, iniciándose tratamiento oncoespecífico.
Citas
(1) Ang CS, Kelley RK, Choti MA, David P, Cosgrove DP, Chou JF, et al. Clinicopathologic characteristics and survival outcomes of patients with fibrolamellar carcinoma: data from the fibrolamellar carcinoma consortium. Gastrointest Cancer Res 2013; 6:3-9.
(2) Honeyman JN, Simon EP, Robine N, Chiaroni-Clarke R, Darcy DG, Lim IIP, et al. Detection of a recurrent DNAJB1-PRKACA chimeric transcript in fibrolamellar hepatocellular carcinoma. Science 2014; 343(6174):1010-4.
(3) Cornella H, Alsinet C, Sayols S, Zhang Z, Hao K, Cabellos L, et al. Gastroenterology 2015; 148(4):806-18.e10.
(4) Tomasini MD, Wang Y, Karamafrooz A, Karamafrooz A , Li G, Beuming T, et al. Conformational Landscape of the PRKACA-DNAJB1 Chimeric Kinase, the Driver for Fibrolamellar Hepatocellular Carcinoma. Sci Rep 2018; 8(1):720.
(5) Graham RP, Jin L, Knutson DL, Kloft-Nelson SM, Greipp PT, Waldburger N, et al. DNAJB1-PRKACA is specific for fibrolamellar carcinoma. Mod Pathol 2015; (6):822-9.
(6) Eggert T, McGlynn KA, Duffy A, Manns MP, Greten TF, Altekruse SF. Fibrolamellar hepatocellular carcinoma in the USA, 2000-2010: a detailed report on frequency, treatment and outcome based on the surveillance, epidemiology, and end results database. United European Gastroenterol J 2013; 1:351-7.
(7) Meriggi F, Forni E. [Surgical therapy of hepatic fibrolamellar carcinoma]. Ann Ital Chir 2007; 78:53-8.
(8) Mafeld S, French J, Tiniakos D, Haugk B, Manas D, Litlle P, et al. Fibrolamellar hepatocellular carcinoma: treatment with yttrium-90 and subsequent surgical resection. Cardiovasc Intervent Radiol 2018; 41(5):816-20.
(9) Lafaro KJ, Pawlik TM. Fibrolamellar hepatocellular carcinoma: current clinical perspectives. J Hepatocell Carcinoma 2015; 2:151-7.
(10) Butte JM, Waugh E, Meneses M, Pruzzo R, Carvallo C, Redondo F, et al. Carcinoma hepatocelular variedad fibrolamelar metastásico en menores de 20 años: reporte de 2 casos tratados con intención curativa y revisión de la literatura. Rev méd Chile 2009; 137(3):394-400.
(11) Kassahun WT. Contemporary management of fibrolamellar hepatocellular carcinoma: diagnosis, treatment, outcome, prognostic factors, and recent developments. World J Surg Oncol 2016; 14(1):151.
(12) Berman MA, Burnham JA, Sheahan DG. Fibrolamellar carcinoma of the liver: an inmunohistochemical study of nineteen cases and review of the literature. Hum Pathol 1988; 19:784-94.
(13) Ganeshan D, Szklaruk J, Kundra V, Kaseb V, Rashid A, Elsayes KM. Imaging features of fibrolamellar hepatocellular carcinoma. AJR Am J Roentgenol 2014; 202(3):544-52.
(14) Ichikawa T, Federle MP, Grazioli L, Madariaga J, Nalesnik M, Marsh W. Fibrolamellar hepatocellular carcinoma: imaging and pathologic findings in 31 recent cases. Radiology 1999; 213:352-61.
(15) Friedman AC, Lichtenstein JE, Goodman Z, Fishman EK, Siegelman SS, Dachman AH. Fibrolamellar hepatocellular carcinoma. Radiology 1985; 157:583-7.
(16) Blachar A, Federle MP, Ferris JV, Lacomis JM, Waltz JS, Amfield DR, et al. Radiologists performance in the diagnosis of liver tumors with central scars by using specific CT criteria. Radiolgy 2002; 223:532-53.
(17) Crawford JM. El hígado y las vías biliares. En: Kumar V, Abbas A, Fausto N. Robbins y Cotran. Patología estructural y funcional. 7ª ed. Madrid: Elsevier, 2005:881-942.
(18) El-Serag HB, Davila JA. Is fibrolamellar carcinoma different from hepatocelullar carcinoma? A US population-based study. Hepatology 2004; 39:798-803.
(19) Ichikawa T, Federle MP, Grazioli L, Marsh W. Fibrolamellar hepatocellular carcinoma: pre-and posttherapy evaluation with CT and MR imaging. Radiology 2000; 217:145-51.
(20) Epstein BE, Pajak TF, Haulk TL, Herpst JM, Order SE, Abrams RA. Metastatic nonresectable fibrolamellar hepatoma: prognostic features and natural history. Am J Clin Oncol 1999; 22:22-8.
(21) Malouf GG, Brugieres L, Le Deley MC, Faivre S, Fabre M, Paradis V, et al. Pure and mixd fibrolamellar hepatocellular carcinomas differ in natural history and prognosis after complete surgical resection. Cancer 2012; 118:4981-90.
(22) McLarney JK, Rucker PT, Bender GN, Goodman DZ, Kashiyani N, Ros PR. Fibrolamellar carcinoma of the liver: radiologic-pathologic correlation. Radiographics 1999; 19:453-71.
(23) Mavros MN, Mayo SC, Hyder O, Pawlik TM. A systematic review: treatment and prognosis of patients with fibrolamellar hepatocelullar carcinoma. J AM Coll Surg 2012; 215:820-30.
(24) Kaseb AO, Shama M, Sahin IH, Nooka A, Hassabo HM, Vauthey JN, et al. Prognostic indicators and treatment outcome in 94 cases of fibrolamellar hepatocellular carcinoma. Oncology 2013; 85:197-203.
(25) Chakrabarti S, Tella SH, Kommalapati A, Huffman BM, Yadav S, Riaz IB, et al. Clinicopathological features and outcomes of fibrolamellar hepatocellular carcinoma. J Gastrointest Oncol 2019; 10(3):554-61.
(2) Honeyman JN, Simon EP, Robine N, Chiaroni-Clarke R, Darcy DG, Lim IIP, et al. Detection of a recurrent DNAJB1-PRKACA chimeric transcript in fibrolamellar hepatocellular carcinoma. Science 2014; 343(6174):1010-4.
(3) Cornella H, Alsinet C, Sayols S, Zhang Z, Hao K, Cabellos L, et al. Gastroenterology 2015; 148(4):806-18.e10.
(4) Tomasini MD, Wang Y, Karamafrooz A, Karamafrooz A , Li G, Beuming T, et al. Conformational Landscape of the PRKACA-DNAJB1 Chimeric Kinase, the Driver for Fibrolamellar Hepatocellular Carcinoma. Sci Rep 2018; 8(1):720.
(5) Graham RP, Jin L, Knutson DL, Kloft-Nelson SM, Greipp PT, Waldburger N, et al. DNAJB1-PRKACA is specific for fibrolamellar carcinoma. Mod Pathol 2015; (6):822-9.
(6) Eggert T, McGlynn KA, Duffy A, Manns MP, Greten TF, Altekruse SF. Fibrolamellar hepatocellular carcinoma in the USA, 2000-2010: a detailed report on frequency, treatment and outcome based on the surveillance, epidemiology, and end results database. United European Gastroenterol J 2013; 1:351-7.
(7) Meriggi F, Forni E. [Surgical therapy of hepatic fibrolamellar carcinoma]. Ann Ital Chir 2007; 78:53-8.
(8) Mafeld S, French J, Tiniakos D, Haugk B, Manas D, Litlle P, et al. Fibrolamellar hepatocellular carcinoma: treatment with yttrium-90 and subsequent surgical resection. Cardiovasc Intervent Radiol 2018; 41(5):816-20.
(9) Lafaro KJ, Pawlik TM. Fibrolamellar hepatocellular carcinoma: current clinical perspectives. J Hepatocell Carcinoma 2015; 2:151-7.
(10) Butte JM, Waugh E, Meneses M, Pruzzo R, Carvallo C, Redondo F, et al. Carcinoma hepatocelular variedad fibrolamelar metastásico en menores de 20 años: reporte de 2 casos tratados con intención curativa y revisión de la literatura. Rev méd Chile 2009; 137(3):394-400.
(11) Kassahun WT. Contemporary management of fibrolamellar hepatocellular carcinoma: diagnosis, treatment, outcome, prognostic factors, and recent developments. World J Surg Oncol 2016; 14(1):151.
(12) Berman MA, Burnham JA, Sheahan DG. Fibrolamellar carcinoma of the liver: an inmunohistochemical study of nineteen cases and review of the literature. Hum Pathol 1988; 19:784-94.
(13) Ganeshan D, Szklaruk J, Kundra V, Kaseb V, Rashid A, Elsayes KM. Imaging features of fibrolamellar hepatocellular carcinoma. AJR Am J Roentgenol 2014; 202(3):544-52.
(14) Ichikawa T, Federle MP, Grazioli L, Madariaga J, Nalesnik M, Marsh W. Fibrolamellar hepatocellular carcinoma: imaging and pathologic findings in 31 recent cases. Radiology 1999; 213:352-61.
(15) Friedman AC, Lichtenstein JE, Goodman Z, Fishman EK, Siegelman SS, Dachman AH. Fibrolamellar hepatocellular carcinoma. Radiology 1985; 157:583-7.
(16) Blachar A, Federle MP, Ferris JV, Lacomis JM, Waltz JS, Amfield DR, et al. Radiologists performance in the diagnosis of liver tumors with central scars by using specific CT criteria. Radiolgy 2002; 223:532-53.
(17) Crawford JM. El hígado y las vías biliares. En: Kumar V, Abbas A, Fausto N. Robbins y Cotran. Patología estructural y funcional. 7ª ed. Madrid: Elsevier, 2005:881-942.
(18) El-Serag HB, Davila JA. Is fibrolamellar carcinoma different from hepatocelullar carcinoma? A US population-based study. Hepatology 2004; 39:798-803.
(19) Ichikawa T, Federle MP, Grazioli L, Marsh W. Fibrolamellar hepatocellular carcinoma: pre-and posttherapy evaluation with CT and MR imaging. Radiology 2000; 217:145-51.
(20) Epstein BE, Pajak TF, Haulk TL, Herpst JM, Order SE, Abrams RA. Metastatic nonresectable fibrolamellar hepatoma: prognostic features and natural history. Am J Clin Oncol 1999; 22:22-8.
(21) Malouf GG, Brugieres L, Le Deley MC, Faivre S, Fabre M, Paradis V, et al. Pure and mixd fibrolamellar hepatocellular carcinomas differ in natural history and prognosis after complete surgical resection. Cancer 2012; 118:4981-90.
(22) McLarney JK, Rucker PT, Bender GN, Goodman DZ, Kashiyani N, Ros PR. Fibrolamellar carcinoma of the liver: radiologic-pathologic correlation. Radiographics 1999; 19:453-71.
(23) Mavros MN, Mayo SC, Hyder O, Pawlik TM. A systematic review: treatment and prognosis of patients with fibrolamellar hepatocelullar carcinoma. J AM Coll Surg 2012; 215:820-30.
(24) Kaseb AO, Shama M, Sahin IH, Nooka A, Hassabo HM, Vauthey JN, et al. Prognostic indicators and treatment outcome in 94 cases of fibrolamellar hepatocellular carcinoma. Oncology 2013; 85:197-203.
(25) Chakrabarti S, Tella SH, Kommalapati A, Huffman BM, Yadav S, Riaz IB, et al. Clinicopathological features and outcomes of fibrolamellar hepatocellular carcinoma. J Gastrointest Oncol 2019; 10(3):554-61.
Publicado
2020-04-22
Cómo citar
1.
Ferreira Pozzi M, Refreschini Ruiz D, Wagner Taustanowski G, Vaucher Rivero A, González González D. Hepatocarcinoma fibrolamelar. Rev. Méd. Urug. [Internet]. 22 de abril de 2020 [citado 24 de diciembre de 2024];36(2):186-90. Disponible en: http://www2.rmu.org.uy/ojsrmu311/index.php/rmu/article/view/534
Sección
Casos Clínicos
