Outcomes of Patients with Scirrhous Hepatocellular Carcinoma: Insights from the National Cancer Database

Farooq, Ayesha; Merath, Katiuscha; Paredes, Anghela Z.; Wu, Lu; Tsilimigras, Diamantis I.; Hyer, J. Madison; Sahara, Kota; Mehta, Rittal; Beal, Eliza W.; Pawlik, Timothy M.

doi:10.1007/s11605-019-04282-1

Cited by 7 publications

(4 citation statements)

References 25 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: 71%

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: 71%

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

Chen

Shi

et al. 2022

Front. Oncol.

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“…The survival outcome for this subtype remains to be further delineated. Overall survival of patients was found comparable with non-scirrhous HCC in some studies [36,37] , while both better [38][39][40] and worse [35] survival outcomes were also reported. Furthermore, scirrhous HCC was associated with less frequent HBV infection, lower serum AFP level and less liver cirrhosis when compared with conventional HCC [37] .…”

Section: Scirrhous Hccmentioning

confidence: 81%

“…Scirrhous HCC shows peculiar histology with small oval cells arranged in nests or trabecular among an abundant fibrous stroma [35] . It comprises 0.19% of all HCC from the National Cancer Database from 2004-2015 [36] . The survival outcome for this subtype remains to be further delineated.…”

Section: Scirrhous Hccmentioning

confidence: 99%

An update on the histological subtypes of hepatocellular carcinoma

Lo¹

2019

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Hepatocellular carcinoma (HCC) is one of the top-ranking cancers worldwide and in Southeast Asia. The high propensity for tumor recurrence, distant metastasis and chemoresistance remain major hurdles in the treatment of HCC. With advances on genetics and genomics research, molecular targeted therapies are emerging as a hope for better disease control. On the histological perspective, microscopic review of clinical samples has led to subclassification of HCC and establishment of new entities. In this review, latest understanding on macrotrabecular-massive HCC, steatohepatitic HCC, lymphocyte-rich HCC, scirrhous HCC, fibrolamellar carcinoma and combined hepatocellular-cholangiocarcinoma will be discussed, emphasizing on the clinical relevance of these pathological entities. Further delineation of the histological, immunohistochemical, molecular and biological phenotypes of primary liver cancer would to further enhance an integrated morphological-molecular classification that better predicts clinical outcome and guides clinical management.

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“…Scirrhous hepatocellular carcinoma (S-HCC) is an uncommon subtype of HCC, representing approximately 4.6% of all cases ( 1 ), which develops primarily in livers affected by hepatitis or cirrhosis ( 2 ). Due to extensive tumor fibrosis, radiologists often misclassify this type of tumor as intrahepatic cholangiocarcinoma or a mixed form of hepatocellular cholangiocarcinoma ( 1 , 3 , 4 ). Although S-HCC has a hepatocellular structure, more than half of tumors show abundant and diffuse intratumoral fibrosis ( 5 ).…”

Section: Introductionmentioning

confidence: 99%

Favorable outcome of immunotherapy in a rare subtype of hepatocellular carcinoma: a case report and literature review

Archwamety,

Kunapinun,

Sirinvaravong

et al. 2024

Front. Oncol.

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Scirrhous hepatocellular carcinoma (S-HCC) represents an uncommon subtype of HCC. During radiological evaluation this unique subtype is frequently mistaken as cholangiocarcinoma, fibrolamellar HCC, or metastatic adenocarcinoma. Here, we present the case of a 50-year-old woman with a large hepatic mass. A triple-phase computed tomography of the liver revealed an arterial enhancing lesion without portovenous washout at hepatic segment 4a/8. The liver biopsy showed hepatocellular characteristics and was positive for Hep Par 1, CK7, CK19, Arginase 1 and CEA, indicating atypical S-HCC. This patient had achieved tumor control with combined treatment with atezolizumab plus bevacizumab and was then treated with lenvatinib after tumor progression. The patient died 15 months after the initial diagnosis.

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Outcomes of Patients with Scirrhous Hepatocellular Carcinoma: Insights from the National Cancer Database

Cited by 7 publications

References 25 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

An update on the histological subtypes of hepatocellular carcinoma

Favorable outcome of immunotherapy in a rare subtype of hepatocellular carcinoma: a case report and literature review

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