A Resected Case of Fibrolamellar Carcinoma in a 24 Years Old Woman: A Case Report and Review of Japanese Cases

Yasuda, Hiroshi; Yamagata, Kenji; Hifumi, Michio; Hayashi, Kouji; Hirata, Toshihiko; Terakura, Hirotsugu; Yamane, Takaaki; Kawaguchi, Tetsu; Fukuda, Seiji; Matsukane, Hidenobu

doi:10.5833/jjgs.37.1645

Cited by 5 publications

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“…In our case, the CT image of the tumor showed calcification and the central scar exhibited hypointensity on the T1WI MRI. FL-HCC is primarily detected in children as a large mass exceeding 7–8 cm [ 15 ]. In the present case, preoperative diagnosis was challenging because the size was smaller than in previous cases.…”

Section: Discussionmentioning

confidence: 99%

Fibrolamellar hepatocellular carcinoma: a case report and gene analysis

Watanabe,

Harimoto,

Saito

et al. 2023

surg case rep

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Background Fibrolamellar hepatocellular carcinoma (HCC) (FL-HCC) is rare in Japan. FL-HCC develops in young patients with no history of cirrhosis and tends to manifest lymphatic metastasis with clinical features similar to those of HCC. We present a case of FL-HCC in a young male patient. Case presentation A 14-year-old male patient underwent abdominal computed tomography (CT) to diagnose appendicitis, wherein a hepatic tumor was detected. Dynamic enhanced CT revealed a 35-mm solid tumor, which contrasted at the early phase of dynamic enhanced study of the right hepatic segments, with occlusion of the right portal vein. We performed right hepatectomy for these lesions. The patient experienced a single lymphatic recurrence on the hepatoduodenal ligament 12 months after the initial surgery. We performed lymphadenectomy for the recurrent tumor. We performed RNA sequencing (RNA-seq) and targeted DNA sequencing of the resected specimens (primary tumor, lymphatic metastasis, and normal liver). RNA-seq detected DNAJB1-PRKACA in both primary and metastatic lesions as previously reported. Furthermore, The Cancer Genome Atlas (TCGA) database was used to compare other gene expressions in this case with those of previously reported cases of FL-HCC and HCC in young patients. Principal component analysis of differentially expressed genes in the top 10% revealed that the gene expression in our case was similar to that of previous FL-HCC cases but was a different cluster from that in HCC cases in young patients. Mutational analysis did not detect any somatic mutations associated with carcinogenesis, including previously reported mutations (Kastenhuber et al. in Proc Natl Acad Sci USA 114: 13076–84, 2017). Conclusion We encountered a case of FL-HCC, a rare hepatic tumor in an adolescent patient, and evaluated the genetic background. Our findings could contribute to the elucidation of the mechanisms underlying carcinogenesis and progression in patients with FL-HCC and thereby contribute to the development of new therapeutic strategies in the future that may improve patient prognosis.

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Section: Discussionmentioning

confidence: 99%

Fibrolamellar hepatocellular carcinoma: a case report and gene analysis

Watanabe,

Harimoto,

Saito

et al. 2023

surg case rep

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“…Fibrolamellar hepatocellular carcinoma (FLC) is a rare form of primary liver cancer and is known to occur predominantly in young adults who typically have normal background liver functions and serum α-fetoprotein (AFP) values. It accounts for 1–2% of primary liver cancer in Europe and the United States [ 2 ], but the incidence in Japan is low [ 3 , 4 ].…”

Section: Introductionmentioning

confidence: 99%

Immunohistochemical detection of procalcitonin in fibrolamellar hepatocellular carcinoma

Matsumoto

Kikuchi

Hara

et al. 2021

Clin J Gastroenterol

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A 25-year-old woman with fever and epigastric pain was referred to our hospital. Blood examination showed significant liver dysfunction, markedly high C-reactive protein (CRP 19.1 mg/dL) and procalcitonin (48.3 ng/mL) levels. Dynamic computed tomography showed a tumor approximately 120 mm in size in the right lobe of the liver, but with no abscess formation. The patient was hospitalized and started on antibiotics; her CRP level improved, but the procalcitonin level did not decrease. Histopathological examination of the liver tumor biopsy revealed fibrolamellar hepatocellular carcinoma (FLC). Positive staining of the FLC with an anti-procalcitonin antibody suggested the production of procalcitonin.

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Typical fibrolamellar hepatocellular carcinoma in a Japanese boy: report of a case

et al. 2013

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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.

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A Resected Case of Fibrolamellar Carcinoma in a 24 Years Old Woman: A Case Report and Review of Japanese Cases

Cited by 5 publications

References 0 publications

Fibrolamellar hepatocellular carcinoma: a case report and gene analysis

Fibrolamellar hepatocellular carcinoma: a case report and gene analysis

Immunohistochemical detection of procalcitonin in fibrolamellar hepatocellular carcinoma

Typical fibrolamellar hepatocellular carcinoma in a Japanese boy: report of a case

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