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

Matsuda, Masanori; Amemiya, Hidetake; Kawaida, Hiromichi; Okamoto, Hiroyuki; Hosomura, Naohiro; Asakawa, Masami; Sano, Katsuhiro; Ichikawa, Tomoaki; Nakazawa, Tadao; Fujii, Hideki

doi:10.1007/s00595-013-0653-y

Cited by 5 publications

(3 citation statements)

References 29 publications

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“…In one case report of a pediatric FLC patient the tumor was considered to not show uptake of gadoxetic acid in HBP [ 19 ]. In a larger cohort of 37 FLCs only one gadoxetic acid-enhanced MRI was performed, and the tumor was uniformly hypointense in HBP compared with background liver parenchyma [ 14 ].…”

Section: Discussionmentioning

confidence: 99%

Imaging features of fibrolamellar hepatocellular carcinoma in gadoxetic acid-enhanced MRI

et al. 2018

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BackgroundFibrolamellar hepatocellular carcinoma (FLC) is a rare malignancy occurring in young patients without cirrhosis. Objectives of our study were to analyze contrast material uptake in hepatobiliary phase imaging (HBP) in gadoxetic acid-enhanced liver MRI in patients with FLC and to characterize imaging features in sequence techniques other than HBP.MethodsIn this retrospective study on histology-proven FLC, contrast material uptake in HBP was quantitatively assessed by calculating the corrected FLC enhancement index (CEI) using mean signal intensities of FLC and lumbar muscle on pre-contrast imaging and HBP, respectively. Moreover, enhancement patterns in dynamic contrast-enhanced MRI and relative signal intensities compared with background liver parenchyma were determined by two radiologists in consensus for HBP, diffusion-weighted imaging using high b-values (DWI), and T2 and T1 weighted pre-contrast imaging.ResultsIn 6 of 13 patients with FLC gadoxetic acid-enhanced liver MRI was available. The CEI suggested presence of HBP contrast material uptake in all FLCs. A mean CEI of 1.35 indicated FLC signal increase of 35% in HBP compared with pre-contrast imaging. All FLCs were hypointense in HBP compared with background liver parenchyma. Three of 6 FLCs had arterial hyperenhancement and venous wash-out. In DWI and T2 weighted imaging, 5 of 6 FLCs were hyperintense. In T1 weighted imaging, 5 of 6 FLCs were hypointense.ConclusionHepatobiliary uptake of gadoxetic acid was quantitatively measurable in all FLCs investigated in our study. The observation of hypointensity of FLCs in HBP compared with background liver parenchyma emphasizes the role of gadoxetic acid-enhanced liver MRI for non-invasive diagnosis of FLC and its importance in the diagnostic work-up of indeterminate liver lesions.

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Section: Discussionmentioning

confidence: 99%

Imaging features of fibrolamellar hepatocellular carcinoma in gadoxetic acid-enhanced MRI

et al. 2018

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“…His AFP level was normal. In contrast to conventional HCC, AFP levels in FL-HCC are usually normal, although elevated levels have been reported in up to 25%percnt; of patients with FL-HCC [ 1 , 10 ].…”

Section: Discussionmentioning

confidence: 99%

Metastatic Fibrolamellar Hepatocellular Carcinoma to the Pancreas

Villa¹,

Pannala²,

Faigel³

et al. 2015

Case Rep Gastroenterol

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Fibrolamellar hepatocellular carcinoma (FL-HCC) is a rare variant of hepatocellular carcinoma, usually presenting in the younger population (<40 years) without underlying liver disease. Although it has a better prognosis than hepatocellular carcinoma, it has a high rate of recurrence months to years after primary resection. While sites of recurrence usually involve the liver, regional lymph nodes, peritoneum, and lung, metastasis to the pancreas is extremely rare, with only 2 other cases reported in the literature. We present the case of a 46-year-old patient with metastatic FL-HCC to the pancreas 30 years after diagnosis and 26 years since his last resected liver recurrence.

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“…These markers have been associated with disease burden [ 8 , 9 , 10 , 11 , 12 ]. Serum α fetoprotein (AFP) may be considered during the clinical and laboratory work-up, but it is elevated in only 10% vs. 60% of HCC with classic morphology [ 13 , 14 ]. Clinically, individuals harboring FL-HCC have similar symptoms to HCC of classic type, but may also present with two unusual phenotypes, including gynecomastia and Budd-Chiari syndrome [ 15 , 16 , 17 , 18 , 19 ].…”

Section: Introductionmentioning

confidence: 99%

Hepatocellular Carcinoma, Fibrolamellar Variant: Diagnostic Pathologic Criteria and Molecular Pathology Update. A Primer

Sergi

2015

Diagnostics

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Fibrolamellar hepatocellular carcinoma (FL-HCC) is generally a fairly rare event in routine pathology practice. This variant of hepatocellular carcinoma (HCC) is peculiarly intriguing and,in addition, poorly understood. Young people or children are often the target individuals with this type of cancer. Previously, I highlighted some pathology aspects of FL-HCC, but in this review, the distinctive clinico-pathologic features of FL-HCC and the diagnostic pathologic criteria of FL-HCC are fractionally reviewed and expanded upon. Further, molecular genetics update data with reference to this specific tumor are particularly highlighted as a primer for general pathologists and pediatric histopathologists. FL-HCC may present with metastases, and regional lymph nodes may be sites of metastatic spread. However, peritoneal and pulmonary metastatic foci have also been reported. To the best of our knowledge, FL-HCC was initially considered having an indolent course, but survival outcomes have recently been updated reconsidering the prognosis of this tumor. Patients seem to respond well to surgical resection, but recurrences are common. Thus, alternative therapies, such as chemotherapy and radiation, are ongoing. Overall, it seems that this aspect has not been well-studied for this variant of HCC and should be considered as target for future clinical trials. Remarkably, FL-HCC data seem to point to a liver neoplasm of uncertain origin and unveiled outcome. A functional chimeric transcript incorporating DNAJB1 and PRKACA was recently added to FL-HCC. This sensational result may give remarkable insights into the understanding of this rare disease and potentially provide the basis for its specific diagnostic marker. Detection of DNAJB1-PRKACA seems to be, indeed, a very sensitive and specific finding in supporting the diagnosis of FL-HCC. In a quite diffuse opinion, prognosis of this tumor should be reconsidered following the potentially mandatory application of new molecular biological tools.

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

Cited by 5 publications

References 29 publications

Imaging features of fibrolamellar hepatocellular carcinoma in gadoxetic acid-enhanced MRI

Imaging features of fibrolamellar hepatocellular carcinoma in gadoxetic acid-enhanced MRI

Metastatic Fibrolamellar Hepatocellular Carcinoma to the Pancreas

Hepatocellular Carcinoma, Fibrolamellar Variant: Diagnostic Pathologic Criteria and Molecular Pathology Update. A Primer

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