Cytologic features of fibrolamellar carcinoma with mucin production: A rare variant of combined hepatocellular‐cholangiocarcinoma

Rosa, Marilin; Mohammadi, Amir

doi:10.1002/dc.22931

Cited by 3 publications

(1 citation statement)

References 15 publications

(36 reference statements)

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“…Cytology may play a major role in the differential diagnosis of hepatic masses [ 50 , 51 , 52 , 53 , 54 ]. Cytologically, FL-HCC is particularly impressive.…”

Section: Gross Anatomy and Microscopymentioning

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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“…Cytology may play a major role in the differential diagnosis of hepatic masses [ 50 , 51 , 52 , 53 , 54 ]. Cytologically, FL-HCC is particularly impressive.…”

Section: Gross Anatomy and Microscopymentioning

confidence: 99%

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

Sergi

2015

Diagnostics

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Combined Hepatocellular-Cholangiocarcinoma

Zimmermann¹

2016

Tumors and Tumor-Like Lesions of the Hepatobiliary Tract

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Combined hepatocellular-cholangiocarcinoma (CHCC-C) is defined as a tumor that contains unequivocal, intimately mixed elements of both hepatocellular carcinoma and cholangiocarcinoma, resulting in a biphenotypic histologic picture. CHCC-C should not be confounded with collision tumors or rare hepatic mixed tumors. This is a rare liver malignancy of older individuals, probably accounting for less than 5 % of hepatic malignancies. The neoplasm develops in both non-cirrhotic and cirrhotic livers. Etiologic factors include hepatitis virus infections and alcoholic liver disease. CHCC-C usually presents as a solitary tumor mass. Similar to hepatocellular carcinoma, the tumor tends to invade the portal vein system and bile ducts. Based on distinct histologic patterns, several types of CHCC-C are distinguished in the WHO classification, including a classical type and a type with stem cell features, the latter further subdivided into at least four subtypes. Rare variants of CHCC-C contain sarcomatoid components, or the HCC component is that of fibrolamellar hepatocellular carcinoma. CHCC-Cs are usually highly aggressive lesions with a high rate of metastatic spread.

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Combined Hepatocellular-Cholangiocarcinoma

Zimmermann¹

2016

Tumors and Tumor-Like Lesions of the Hepatobiliary Tract

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Cytologic features of fibrolamellar carcinoma with mucin production: A rare variant of combined hepatocellular‐cholangiocarcinoma

Cited by 3 publications

References 15 publications

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

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

Combined Hepatocellular-Cholangiocarcinoma

Combined Hepatocellular-Cholangiocarcinoma

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