Clinical manifestations and outcomes of patients with scirrhous hepatocellular carcinoma

Huang, Shang‐Chin; Liao, Shutsung; Su, Tung‐Hung; Jeng, Yung‐Ming; Kao, Jia‐Horng

doi:10.1007/s12072-021-10146-1

Cited by 5 publications

(10 citation statements)

References 22 publications

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“…As another HCC subtype rich in stromal components, scirrhous carcinoma shared almost the same macroscopical clinical characteristics and prognoses with classic HCC, which was also supported by other researchers (19,21,(31)(32)(33). Interestingly, both the two rare subtypes might present unusual paraneoplastic manifestations: hyperammonemic encephalopathy (HAE) occurred in patients with fibrolamellar carcinoma, and hypercalcemia was found in scirrhous carcinoma sufferers (34)(35)(36)(37).…”

Section: Discussionsupporting

confidence: 72%

“…Therefore, more attention should still be paid to developing better therapeutic strategies to further improve the prognoses of HCC patients. In general, classic HCC is characterized by a lack of fibrous stroma, but there exist two rare subtypes of fibrolamellar and scirrhous carcinoma with abundant fibrous stroma (19)(20)(21)(22)(23)(24)(25). As the second common rare subtype in our cohort, fibrolamellar carcinoma was first described by Edmondson in 1956 (26) and named by Craig in 1980 (27).…”

Section: Discussionmentioning

confidence: 83%

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Epidemiological and Clinical Characteristics of Five Rare Pathological Subtypes of Hepatocellular Carcinoma

Chen

Shi

et al. 2022

Front. Oncol.

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BackgroundHepatocellular carcinoma (HCC) is a highly heterogeneous tumor with several rare pathological subtypes and which is still poorly understood. This study aimed to describe the epidemiological and clinical spectrum of five rare HCC subtypes and develop a competing risk nomogram for cancer-specific survival prediction.MethodsThe study cohort was recruited from the Surveillance, Epidemiology, and End Results database. The clinicopathological data of 50,218 patients histologically diagnosed with classic HCC and five rare subtypes (ICD-O-3 Histology Code = 8170/3-8175/3) between 2004 and 2018 were reviewed. The annual percent change (APC) was calculated utilizing Joinpoint regression. The nomogram was developed based on multivariable competing risk survival analyses. Akaike information criterion, Bayesian information criterion, C-index, calibration curve, and area under the receiver operating characteristic curve were obtained to evaluate the prognostic performance. A decision curve analysis was introduced to examine the clinical value of the models.ResultsDespite scirrhous carcinoma, which showed a decreasing trend (APC = -6.8%, P = 0.025), the morbidity of other rare subtypes remained stable from 2004 to 2018. The incidence-based mortality was plateau in all subtypes during the period. Clear cell carcinoma is the most common subtype (n = 551, 1.1%), followed by subtypes of fibrolamellar (n = 241, 0.5%), scirrhous (n = 82, 0.2%), spindle cell (n = 61, 0.1%), and pleomorphic (n = 17, ~0%). The patients with fibrolamellar carcinoma were younger and more likely to have a non-cirrhotic liver and better prognoses. Scirrhous carcinoma shared almost the same macro-clinical characteristics and outcomes as the classic HCC. Clear cell carcinoma tended to occur in the Asia-Pacific elderly male population, and more than half of them were large HCC (Size>5cm). Sarcomatoid (including spindle cell and pleomorphic) carcinoma was associated with a larger tumor size, poorer differentiation, and more dismal prognoses. The pathological subtype, T stage, M stage, surgery, alpha-fetoprotein, and cancer history were confirmed as the independent predictors in patients with rare subtypes. The nomogram showed good calibration, discrimination, and net benefits in clinical practice.ConclusionThe rare subtypes had unique clinicopathological features and biological behaviors compared with the classic HCC. Our findings could provide a valuable reference for clinicians. The constructed nomogram could predict the prognoses with good performance, which is meaningful to individualized management.

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Section: Discussionsupporting

confidence: 72%

Section: Discussionmentioning

confidence: 83%

Epidemiological and Clinical Characteristics of Five Rare Pathological Subtypes of Hepatocellular Carcinoma

Chen

Shi

et al. 2022

Front. Oncol.

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“… 4 , 5 , 14–41 Due to scarcity of publications from the US and Taiwan, National Registry analyses of 161 and 30 patients, respectively, were included in the analysis. 39 , 42 …”

Section: Resultsmentioning

confidence: 99%

“…Twenty-four articles 4 , 5 , 14–16 , 18–27 , 29–33 , 36 , 38 , 41 , 42 provided details on histopathological features of the tumors, summarized in Table 3 .…”

Section: Resultsmentioning

confidence: 99%

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Scirrhous Hepatocellular Carcinoma: Systematic Review and Pooled Data Analysis of Clinical, Radiological, and Histopathological Features

Murtha-Lemekhova¹,

Fuchs²,

Schulz³

et al. 2021

JHC

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Background Aberrant subtypes of hepatocellular carcinoma (HCC) account for 20–30% of all HCCs and habitually present a challenge in diagnosis and treatment. Scirrhous hepatocellular carcinoma (s-HCC) is often misdiagnosed as cholangiocarcinoma, fibrolamellar hepatocellular carcinoma, or metastasis. Methods Electronic databases (PubMed, Web of Knowledge, Google Scholar, Cochrane Library, and WHO International Clinical Trials Registry Platform) were searched for publications on scirrhous hepatocellular carcinoma without date or language restrictions. Quality assessment was performed using a tool proposed by Murad et al for case reports and series. For observational studies, MINORS quality assessment tool was used. This study was registered at PROSPERO (CRD42020212323). Results S-HCC arises in patients with chronic hepatitis (hepatitis B in 60% and hepatitis C in 21%). S-HCC primarily affects men with a mean age of 55.8 years. Serum AFP is elevated above 20IU/mL in 66.7% of the patients. On ultrasound, s-HCC presents as hypoechoic or mosaic pattern lesions (47.6% each) and causes a retraction of the liver surface (70%) when near the capsule. Delayed enhancement of the tumor is evident in 87.0%. On MRI, 65.0% of s-HCCs show a target appearance. Histopathologic pattern is mostly irregular (97.6%). Lesions show a bulging appearance (100%), septae (85.6%) and a central scar (63.5%), and usually lack central necrosis (75%). Immunohistochemistry shows HepPar 1 positivity in 64.6%, CK7 in 40.7%, and EMA in 41.9%. The 5-year overall survival rate estimates 45.2% and 45.5% of the patients experience a recurrence after hepatectomy. Conclusion S-HCC is a rare subtype of HCC primarily arising in hepatitis- or cirrhosis-afflicted livers and incorporates atypical radiological and histopathological HCC features. Despite lower recurrence rates, overall survival of patients with s-HCC is poorer than generally for HCC, underlining the need for individualized treatment. Patients with atypical lesions of the liver should be referred to tertiary hospitals for interdisciplinary assessment and treatment.

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Tumor–matrix interaction induces phenotypic switching in liver cancer cells

et al. 2022

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Clinical manifestations and outcomes of patients with scirrhous hepatocellular carcinoma

Cited by 5 publications

References 22 publications

Epidemiological and Clinical Characteristics of Five Rare Pathological Subtypes of Hepatocellular Carcinoma

Epidemiological and Clinical Characteristics of Five Rare Pathological Subtypes of Hepatocellular Carcinoma

Scirrhous Hepatocellular Carcinoma: Systematic Review and Pooled Data Analysis of Clinical, Radiological, and Histopathological Features

Tumor–matrix interaction induces phenotypic switching in liver cancer cells

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