Mixed hepatocellular and cholangiocarcinoma: a rare tumor with a mix of parent phenotypic characteristics

Bergquist, John R.; Groeschl, Ryan T.; Ivanics, Tommy; Shubert, Christopher R.; Habermann, Elizabeth B.; Kendrick, Michael L.; Farnell, Michael B.; Nagorney, David M.; Truty, Mark J.; Smoot, Rory L.

doi:10.1016/j.hpb.2016.07.006

Cited by 40 publications

(26 citation statements)

References 18 publications

(19 reference statements)

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“…Median overall survival (mOS) of patients stratified by the SEER stage for distant, regionalized, and localized cHCC-HCC was 4 months (95% CI, 3–6), 7 months (95% CI, 5–11), and 20 months (95% CI 16–28), respectively ( p < 0.001), with the difference between regionalised and localized explained by suitability for resection ( 12 ). A similar pattern is seen using TNM staging data from the National Cancer Data Base (NCDB) where mOS was 28.6 m for patients with Stage I disease, 24.2 m for stage II, 7.5 m for stage III and 3.1 m for stage IV ( 67 ).…”

Section: Genetic Characterization and Molecular Biologysupporting

confidence: 53%

“…A meta-analysis (2,19,44,51,53,61). of NCDB cases indicates that transplantation does not result in improved outcome when compared with resection in cHCC-ICC, making a case for careful pre-operative diagnostic assessment to minimize the risk of misdiagnosis with HCC and for the limited supply of donor livers to be more beneficially applied for conditions with better post-transplant outcomes (67,70). Non-surgical treatment options in patients with localized disease include ablation procedures, transarterial (chemo)embolization (TA(C)E), hepatic arterial infusional chemotherapy, radioembolization, and systemic therapy (68,71).…”

Section: Treatmentmentioning

confidence: 99%

“…cHCC-ICC is staged by TNM in a clinical context (as opposed to SEER staging of epidemiological data) using the same staging algorithm as for ICC ( Table 3 ) ( 64 – 66 ). It is difficult to get accurate measures of patient survival without treatment (i.e., the true prognosis) but two large epidemiological datasets from the United States provide some guidance ( 12 , 67 ). Median overall survival (mOS) of patients stratified by the SEER stage for distant, regionalized, and localized cHCC-HCC was 4 months (95% CI, 3–6), 7 months (95% CI, 5–11), and 20 months (95% CI 16–28), respectively ( p < 0.001), with the difference between regionalised and localized explained by suitability for resection ( 12 ).…”

Section: Genetic Characterization and Molecular Biologymentioning

confidence: 99%

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Update on the Genetics of and Systemic Therapy Options for Combined Hepatocellular Cholangiocarcinoma

et al. 2020

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Combined hepatocellular-cholangiocarcinoma (cHCC-ICC) is an uncommon and aggressive form of primary liver cancer. Currently, there are no international guidelines for optimal management. For localized tumors, radical resection represents the preferred treatment option, whereas for advanced tumors, systemic therapies recommended for intrahepatic cholangiocarcinoma (ICC) and hepatocellular carcinoma (HCC) are often selected. Emerging information from comparative cohort studies, genomic and transcriptomic data sets are starting to build a case for rationalized approaches to systemic treatment in the advanced setting specific to cHCC-ICC.

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Section: Genetic Characterization and Molecular Biologysupporting

confidence: 53%

Section: Treatmentmentioning

confidence: 99%

Section: Genetic Characterization and Molecular Biologymentioning

confidence: 99%

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Update on the Genetics of and Systemic Therapy Options for Combined Hepatocellular Cholangiocarcinoma

et al. 2020

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“…Data regarding outcomes, long‐term survival, as well as surgical intervention for HCC‐ICC remain, however, poorly defined. Some studies have reported that HCC‐ICC has a worse prognosis, more lymph node metastasis, and more aggressive behavior compared with HCC and ICC . In fact, while hepatectomy is the principal curative‐intent treatment modality, overall survival (OS) after curative‐intent surgery has been reported to be only about 25 to 32 months .…”

Section: Introductionmentioning

confidence: 99%

Management and outcomes among patients with mixed hepatocholangiocellular carcinoma: A population‐based analysis

Spolverato

Bagante

Tsilimigras

et al. 2018

Journal of Surgical Oncology

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Background We sought to define the management of mixed hepatocellular carcinoma‐intrahepatic cholangiocarcinoma (HCC‐ICC) as well as characterize short‐ and long‐term outcomes of patients with mixed HCC‐ICC. Methods Patients diagnosed with HCC‐ICC, HCC, or ICC between 2004 and 2015 were identified from the National Cancer Data Base using the International Classification of Diseases for Oncology codes. Short‐ and long‐term outcomes were assessed using univariate and multivariate analyses. Results Among 174 454 patients, 86.8% had HCC, 12.1% ICC, and 1.1% HCC‐ICC. The incidence of lymphadenectomy was 55.6% among ICC patients vs 15.1% and 34.2% for HCC and HCC‐ICC patients, respectively (P < 0.001). A 90‐day mortality was comparable among patients with HCC (9.1%), ICC (8.8%), and HCC‐ICC (10.5%) (all P > 0.2). While 42.0% of ICC patients received adjuvant chemotherapy, adjuvant chemotherapy among HCC and HCC‐ICC patients was 13.1% and 27.4%, respectively (P < 0.001). A 5‐year survival was 43.5% (95% CI, 42.5‐44.5), 33.3% (95% CI, 31.4‐35.3), 34.4% (95% CI, 29.1‐39.8) for HCC, ICC, and HCC‐ICC patients, respectively. Conclusion Patients who underwent resection of mixed HCC‐ICC had a prognosis that was comparable to ICC, yet worse than HCC. Utilization of lymphadenectomy and adjuvant therapy were low. HCC‐ICC remains a rare disease with a guarded prognosis that should be treated in a multidisciplinary setting.

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“…The majority of intrahepatic cancers are hepatocellular carcinomas (HCCs) or intrahepatic cholangiocarcinomas (ICCs). The prevalence of hepatocholangiocarcinoma (cHCC-ICC), combining histological features of HCC and ICC, ranges from 1% to 5% of primary hepatic cancers[ 2 ]. In 1949, Allen and Lisa[ 3 ] were the first to describe and classify cHCC-ICC into three subtypes (A, B and C).…”

Section: Introductionmentioning

confidence: 99%

Esophageal metastasis of stem cell-subtype hepatocholangiocarcinoma: Rare presentation of a rare tumor

Salimon¹,

Chapelle²,

Matysiak‐Budnik³

et al. 2018

WJG

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Hepatocholangiocarcinoma (cHCC-ICC) is a rare primary hepatic tumor defined by the presence of histological features of both hepatocellular carcinoma (HCC) and intrahepatic cholangiocarcinoma (ICC). Its prevalence ranges from 1%-5% of all primary liver cancers. We report the case of a 55-year-old cirrhotic male patient admitted to our university hospital for dysphagia, revealing a 10 cm lower-third esophageal metastasis of an unresectable cHCC-ICC with stem-cell features. Computed tomography and abdominal magnetic resonance imaging scans revealed multiple hepatic lesions combining features of both HCC and ICC, associated with synchronous bone metastasis. Histological and immunohistochemical analyses of biopsies from the esophageal lesion and the hepatic tumor confirmed the diagnosis of cHCC-ICC with a stem cell-subtype, according to the World Health Organization classification. After a multidisciplinary meeting, the patient was treated with chemotherapy. He received two cycles of a gemcitabine plus cisplatin regimen before bone progression, and he died 3 mo after the initial diagnosis.

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Mixed hepatocellular and cholangiocarcinoma: a rare tumor with a mix of parent phenotypic characteristics

Abstract: Background: Intrahepatic lesions of mixed hepatocellular (HCC) and intrahepatic cholangiocellular

Cited by 40 publications

References 18 publications

Update on the Genetics of and Systemic Therapy Options for Combined Hepatocellular Cholangiocarcinoma

Update on the Genetics of and Systemic Therapy Options for Combined Hepatocellular Cholangiocarcinoma

Management and outcomes among patients with mixed hepatocholangiocellular carcinoma: A population‐based analysis

Esophageal metastasis of stem cell-subtype hepatocholangiocarcinoma: Rare presentation of a rare tumor

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