Abstract
We report a case of typical fibrolamellar hepatocellular carcinoma (FL-HCC) in a 16-year-old Japanese boy. This is very rare malignancy in Japan. The patient was referred for investigation of a large hepatic tumor and the results of tests for hepatitis B virus surface antigen and hepatitis C virus antibody were negative. Liver function test results and serum alpha-fetoprotein (AFP) levels were normal; however, the prothrombin induced by vitamin K absence/antagonist II was elevated. Computed tomography (CT) showed a large lobulated heterogeneously enhanced tumor in the posterior section of the liver. We diagnosed FL-HCC and performed posterior sectionectomy of the liver. The resected specimen contained a light brown and green tumor with a central fibrous scar, 10.0 cm in diameter. Microscopic and electron microscopic examinations revealed the typical features of FL-HCC. Immunohistochemically, the tumor cells were positive for cytokeratin (CK) 7, but negative for CK19 and AFP. The patient was alive without recurrence 48 months after surgery. Following this case report, we summarize the clinical features of the Japanese cases documented in the literature.
References
Craig JR, Peters RL, Edmondson HA, Omata M. Fibrolamellar carcinoma of the liver: a tumor of adolescents and young adults with distinctive clinico-pathologic features. Cancer. 1980;46:372–9.
Soreide O, Czerniak A, Bradpiece H, Bloom S, Blumgart L. Characteristics of fibrolamellar hepatocellular carcinoma. A study of nine cases and a review of the literature. Am J Surg. 1986;151:518–23.
Fibrolamellar hepatocellular carcinoma. In: Ishak KG, Goodman ZD, Stocker JT, editors. Tumor of the liver and intrahepatic bile ducts. Atlas of tumor pathology. 3rd Series, Fascicle 31. Washington, D.C.: Armed Forces Institute of Pathology; 2001. p. 231–44.
El-Serag HB, Davila JA. Is fibrolamellar carcinoma different from hepatocellular carcinoma? A US population-based study. Hepatology. 2004;39:798–803.
Taniura H, Nagasue N, Kohno H, Kanamori H, Nakamura T, Nagaoka S. A case report of fibrolamellar hepatocellular carcinoma of the liver (in Japanese). Nihon Shoukakigeka Gakkaizassi (Jpn J Gastroenterol Surg). 1987;20:2627–30.
Kohno H, Nagasue N, Taniura H, Nakamura T, Nagaoka S. Fibrolamellar carcinoma of the liver—a case report from Japan and a review of the literature. HPB Surg. 1988;1:77–80.
Hirokawa M, Ryu M, Watanabe K, Watanabe S, Kawakami Y, Yoshida M, et al. A case of fibrolamellar carcinoma of the liver (in Japanese with English abstract). Kanzo (Acta Hepatol Jpon). 1993;34:29–35.
Tanaka J, Baba N, Arii S, Fujita K, Tamura J, Kawakami Y, et al. Typical fibrolamellar hepatocellular carcinoma in Japanese patients: report of two cases. Surg Today. 1994;24:459–63.
Beppu T, Kubota T, Katafuchi S, Honma K, Matsumura F, Takeguchi T, et al. A resected case of fibrolamellar carcinoma in the liver—a distinctive features in diagnostic imagings (in Japanese with English abstract). Kanzo (Acta Hepatol Jpon). 1995;36:601–7.
Hoshino H, Katada N, Nishimura D, Imada J, Morita K, Yoshida N, et al. Case report: fibrolamellar hepatocellular carcinoma in a Japanese woman: a case report and review of Japanese cases. J Gastroenterol Hepatol. 1996;11:551–5.
Sato S, Masuda T, Oikawa H, Satodate R, Suzuki K, Sato S, et al. Bile canaliculi-like lumina in fibrolamellar carcinoma of the liver: a light- and electron-microscopic study and three-dimensional examination of serial sections. Pathol Int. 1997;47:763–8.
Yamazaki H, Kouda E, Kanai T, Ishikawa H, Kuramochi S, Hasegawa I. A case report of fibrolamellar hepatocellular carcinoma (in Japanese with English abstract). Rinsyou Housyasen (Jpn J Clin Radiol). 1998;43:523–6.
Yamaguchi R, Tajika T, Kanda H, Nakanishi K, Kawanishi J. Fibrolamellar carcinoma of the liver. Hepatogastroenterology. 1999;46:1706–9.
Tomimuro T, Kayama H, Funai S, Kou K. A resected case of fibrolamellar carcinoma of the liver (in Japanese). Nihon Gekakei Rengou Gakkaishi. 1999; 24:797–801.
Nojiri T, Mitsumori N, Yoshida T, Shinohara T, Asakura J, Doi N et al. A case of fibrolamellar hepatocellular carcinoma (in Japanese with English abstract). Nihon Shoukakigeka Gakkaizassi (Jpn J Gastroenterol Surg). 2000; 33:1905–9.
Magata S, Kitahara K, Watanabe K, Miyazaki K. Fibrolamellar hepatocellular carcinoma—a case report (in Japanese). Kanzo (Acta Hepatol Jpn). 2001;42:414–9.
Yoshinaga Y, Okazumi S, Takayama W, Makino H, Iwasaki K, Kondou S, et al. A resected case of fibrolamellar hepato cellular carcinoma in a 15 year old woman (in Japanese). Shoukaki Gazou. 2002;4:97–103.
Kanai T, Takabayashi T, Kawano Y, Kuramochi S, Miyazawa N. A case of postoperative recurrence of fibrolamellar hepatocellular carcinoma with increased vitamin B12 binding capacity in a young Japanese female. Jpn J Clin Oncol. 2004;34:346–51.
Yokomizo H, Yamaguchi K, Hifumi M, Hayashi K, Hirata T, Terakura H, et al. A resected case of fibrolamellar carcinoma in a 24 years old woman: a case report and review of Japanese cases (in Japanese with English abstract). Nihon Shoukakigeka Gakkaizassi (Jpn J Gastroenterol Surg). 2004;37:1645–52.
Ooeda Y, Isono T, Yoshioka S, Wakatsuki K, Nukui Y, Takahashi H, et al. A Curative resective case of giant fibrolamellar hepatocellular carcinoma with lymph node metastasis (in Japanese with English abstract). Nihon Shoukakigeka Gakkaizassi (Jpn J Gastroenterol Surg). 2005;38:1732–7.
Hasegawa J, Takano Y, Musha N, Komukai S, Suda K, Fujita K, et al. A resected case of fibrolamellar hepatocellular carcinoma (in Japanese with English abstract). Nihon Rinnsyougeka Gakkai Zasshi (J Jpn Surg Assoc). 2006;67:408–13.
Tanaka Y, Ariizumi S, Katagiri S, Kotera Y, Kato T, Yamamoto M. Fibrolamellar hepatocellular carcinoma in a patient who was given a diagnosis is preoperatively on the basis of magnetic resonance imaging (in Japanese with English abstract). Tokyo Jyoshiikadaigaku Zasshi. 2007;77:83–6.
Kato T, Katagiri S, Ariizumi S, Kotera Y, Takahashi Y, Imai K, et al. A case of retroperitoneal metastasis after resection of fibrolamellar hepatocellular carcinoma (in Japanese with English abstract). Liver Cancer. 2007;13:168–73.
Kato T, Katagiri S, Ariizumi S, Kotera Y, Takahashi Y, Imai K, et al. Three cases of fibrolamellar hepatocellular carcinoma (in Japanese with English abstract). Liver Cancer. 2009;15:78–86.
Ichiki Y, Sugio K, Baba T, Mizukami M, Oga T, Takenoyama M, et al. Mediastinal metastasis from a fibrolamellar hepatocellular carcinoma: report of a case. Surg Today. 2010;40:360–4.
Date Y, Kashida H, Tanaka J, Shiokawa A, Saito K, Morohoshi T. A case of fibrolamellar hepatocellular carcinoma in young woman (in Japanese with English abstract). Liver Cancer. 2009;15:49–56.
Onoe S, Ojima H, Tsurusaki M, Esaki M, Mizuguchi Y, Ban D, et al. A case of fibrolamellar hepatocellular carcinoma followed up for 7 years (in Japanese with English abstract). Liver Cancer. 2009;15:70–7.
Kurahara H, Ueno1 S, Sakoda M, Iino S, Maemura K, Mataki Y et al. A resected case of fibrolamellar hepatocellular carcinoma (in Japanese with English abstract). Kagoshima Daigaku Igakuzasshi (Medical Journal of Kagoshima). 2011;62:55–9.
Cieply B, Zeng G, Proverbs-Singh T, Geller DA, Monga SP. Unique phenotype of hepatocellular cancers with exon-3 mutations in beta-catenin gene. Hepatology. 2009;49:821–31.
Zenali MJ, Tan D, Li W, Dhingra S, Brown RE. Stemness characteristics of fibrolamellar hepatocellular carcinoma: immunohistochemical analysis with comparisons to conventional hepatocellular carcinoma. Ann Clin Lab Sci. 2010;40:126–34.
Abdul Al HM, Wang G, Makhlouf HR, Goodman ZD. Fibrolamellar hepatocellular carcinoma: an immunohistochemical comparison with conventional hepatocellular carcinoma. Int J Surg Pathol. 2010;18:313–8.
Vivekanandan P, Daniel H, Yeh MM, Torbenson M. Mitochondrial mutations in hepatocellular carcinomas and fibrolamellar carcinomas. Mod Pathol. 2010;23:790–8.
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.
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.
Ikai I, Arii S, Okazaki M, Okita K, Omata M, Kojiro M, et al. Report of the 17th nationwide follow-up survey of primary liver cancer in Japan. Hepatol Res. 2007;37:676–91.
Kakar S, Burgart LJ, Batts KP, Garcia J, Jain D, Ferrell LD. Clinicopathologic features and survival in fibrolamellar carcinoma: comparison with conventional hepatocellular carcinoma with and without cirrhosis. Mod Pathol. 2005;18:1417–23.
Stipa F, Yoon SS, Liau KH, Fong Y, Jarnagin WR, D’Angelica M, et al. Outcome of patients with fibrolamellar hepatocellular carcinoma. Cancer. 2006;106:1331–8.
Iwatsuki S, Starzl TE, Sheahan DG, Yokoyama I, Demetris AJ, Todo S, et al. Hepatic resection versus transplantation for hepatocellular carcinoma. Ann Surg. 1991;214:221–8.
Ringe B, Wittekind C, Weimann A, Tusch G, Pichlmayr R. Results of hepatic resection and transplantation for fibrolamellar carcinoma. Surg Gynecol Obstet. 1992;175:299–305.
Pinna AD, Iwatsuki S, Lee RG, Todo S, Madariaga JR, Marsh JW, et al. Treatment of fibrolamellar hepatoma with subtotal hepatectomy or transplantation. Hepatology. 1997;26:877–83.
El-Gazzaz G, Wong W, El-Hadary MK, Gunson BK, Mirza DF, Mayer AD et al. Outcome of liver resection and transplantation for fibrolamellar hepatocellular carcinoma. Transpl Int. 2000;13(Suppl 1):S406–9.
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Masanori Matsuda and his co-authors have no conflicts of interest to report.
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Matsuda, M., Amemiya, H., Kawaida, H. et al. Typical fibrolamellar hepatocellular carcinoma in a Japanese boy: report of a case. Surg Today 44, 1359–1366 (2014). https://doi.org/10.1007/s00595-013-0653-y
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DOI: https://doi.org/10.1007/s00595-013-0653-y