Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2018 Dec 15;57(24):3537-3543.
doi: 10.2169/internalmedicine.1243-18. Epub 2018 Aug 10.

Fibrolamellar Hepatocellular Carcinoma with Multiple Lung Metastases Treated with Multidisciplinary Therapy

Hiroki Tanaka  1   2 , Susumu Hijioka  1   3 , Hiromichi Iwaya  1 , Nobumasa Mizuno  1 , Takamichi Kuwahara  1 , Nozomi Okuno  1 , Ayako Ito  1 , Naosuke Kuraoka  1 , Shinpei Matsumoto  1 , Masahiro Obata  1 , Yusuke Kurita  1 , Muneji Yasuda  1 , Yasuhiro Shimizu  4 , Hiroaki Kuroda  5 , Yozo Sato  6 , Masataka Haneda  7 , Eiichi Sasaki  7 , Yasushi Yatabe  7 , Kazuo Hara  1
Affiliations
Case Reports

Fibrolamellar Hepatocellular Carcinoma with Multiple Lung Metastases Treated with Multidisciplinary Therapy

Hiroki Tanaka et al. Intern Med. .

Abstract

A 20-year old man was diagnosed with fibrolamellar hepatocellular carcinoma (FLHCC) with multiple lung metastases, and chemotherapy with FOLFOX was administered. Contrast enhanced CT after 3 cycles of FOLFOX showed no disease progression. We therefore performed surgical resection and radiofrequency ablation of the liver lesions and lung metastases, after obtaining the patient's informed consent. The liver lesions and lung metastases tested positive for DNAJB1-PRKACA. The treatment for FLHCC with extrahepatic metastasis has not been established; however, in a few cases, good long-term prognoses were obtained with multidisciplinary therapy. We herein report a case of FLHCC with multiple lung metastases that was treated with multidisciplinary therapies.

Keywords: DNAJB1-PRKACA; fibrolamellar hepatocellular carcinoma; multidisciplinary therapy.

PubMed Disclaimer

Figures

Figure 1.
Figure 1.
Abdominal ultrasonography (the lesion in the left lateral segment of the liver). A multi-nodular heterogeneous hypoechoic mass with a cord-like hyperechoic area in the center of the lesion.
Figure 2.
Figure 2.
Contrast enhanced computed tomography. a: The lesion in the left lateral segment of the liver. In the arterial phase, the lesion showed heterogeneous hyperattenuation with the hypoattenuation area in the center of the lesion, and although it showed the gradual washout of contrast medium, it was prolonged in the equilibrium phase. b: The lesion in the segment 4 of the liver. The lesion showed homogeneous hyperattenuation in the arterial phase and hypoattenuation in the equilibrium phase (arrow). c: Multiple metastatic lesions in the lungs.
Figure 3.
Figure 3.
Abdominal MRI (the lesion in the left lateral segment of the liver). a: The tumor was hypointense on T1-weighted images and hyperintense on T2-weighted images. In addition, the center of the lesion was hypointense on T1-weighted images hyperintense on T2-weighted images. b: EOB-MRI. Excluding the center of the lesion, the tumor showed heterogeneous enhancement in the arterial phase and prolonged enhancement in the equilibrium phase. In addition, it was hypointense on the hepatocellular phase.
Figure 4.
Figure 4.
The histopathological analysis (the lesion in the left lateral segment of the liver). a: A well-defined multi-nodular tumor with a central stellate scar. b: The histopathological analysis showed polygonal tumor cells with an eosinophilic cytoplasm surrounded by abundant fibrous stroma. Hematoxylin and Eosin (H&E) staining, × 100. c, d: The stellate scar had prominent fibrous tissue; however, there was a sparse part of the fibrous tissue that was edematous. H&E staining, × 100.
Figure 5.
Figure 5.
Immunohistochemistry (the lesion in the left lateral segment of the liver). a: Hep-par 1 staining, × 200. b: CK7 staining, × 200. c: CD68 staining, × 200.
Figure 6.
Figure 6.
DNAJB1-PRKACA fusion. RT-PCR and direct sequencing showed the fusion of exon 1 from DNAJB1 with exon 2 from PRKACA.
Figure 7.
Figure 7.
Computed tomography after the operation and radiofrequency ablation for lung metastases. There were no obvious lesions in the lungs.
See this image and copyright information in PMC

Similar articles

See all similar articles

Cited by

References

    1. El-Serag HB, Davila JA. Is fibrolamellar carcinoma different from hepatocellular carcinoma? A US population-based study. Hepatology 39: 798-803, 2004. - PubMed
    1. Eggert T, McGlynn KA, Duffy A, et al. . 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 1: 351-357, 2013. - PMC - PubMed
    1. Njei B, Konjeti VR, Ditah I. Prognosis of patients with fibrolamellar hepatocellular carcinoma versus conventional hepatocellular carcinoma: a systematic review and meta-analysis. Gastrointest Cancer Res 7: 49-54, 2014. - PMC - PubMed
    1. Kaseb AO, Shama M, Sahin IH, et al. . Prognostic indicators and treatment outcome in 94 cases of fibrolamellar hepatocellular carcinoma. Oncology 85: 197-203, 2013. - PMC - PubMed
    1. Wahab MA, El Hanafy E, El Nakeeb A, et al. . Clinicopathological features and surgical outcome of patients with fibrolamellar hepatocellular carcinoma (experience with 22 patients over a 15-year period). World J Gastrointest Surg 9: 61-67, 2017. - PMC - PubMed

Publication types

Supplementary concepts