obsolete combined hepatocellular carcinoma and cholangiocarcinoma

Obsolete Combined Hepatocellular Carcinoma and Cholangiocarcinoma

Combined hepatocellular carcinoma and cholangiocarcinoma (cHCC-CCA) is a rare type of liver cancer that was previously referred to as obsolete combined hepatocellular carcinoma and cholangiocarcinoma. This term is no longer used in modern pathology.

Characteristics

• cHCC-CCA is a primary liver carcinoma that exhibits both hepatocytic and cholangiocytic differentiation.
• It accounts for 2-5% of primary liver carcinomas (PLCs).
• The tumor contains two distinct components: a well-differentiated hepatocellular carcinoma component and a well-differentiated intrahepatic cholangiocarcinoma component.

Risk Factors

• Classical risk factors for hepatocellular carcinoma (HCC) also predispose patients to the development of cHCC-CCA.
• These risk factors include chronic liver disease, cirrhosis, and exposure to certain toxins.

Terminology Evolution

• The pathological definition of cHCC-CCA has significantly evolved over time.
• In 2019, the World Health Organization (WHO) classification highlighted that the diagnosis of cHCC-CCA is based on morphological and phenotypical considerations.

References

• [10] Combined hepatocellular-cholangiocarcinoma (or combined hepatocellular carcinoma and cholangiocarcinoma; cHCC-CCA) is a primary liver carcinoma (PLC) defined by the unequivocal presence of both hepatocytic and cholangiocytic differentiation.
• [13] Combined hepatocellular-cholangiocarcinoma (cHCC-CCA) is a tumour that exhibits both hepatocytic and biliary differentiation.
• [14] Combined hepatocellular-cholangiocarcinoma (cHCC-CCA) is a tumour that exhibits both hepatocytic and biliary differentiation.

Diagnostic Tests for Combined Hepatocellular Carcinoma and Cholangiocarcinoma (cHCC-CCA)

The diagnosis of cHCC-CCA can be challenging due to its overlapping features with hepatocellular carcinoma (HCC) and intrahepatic cholangiocarcinoma (ICC). Various diagnostic tests have been employed to aid in the diagnosis of this rare liver cancer.

• Imaging Studies: Imaging modalities such as computed tomography (CT), magnetic resonance imaging (MRI), and ultrasound are commonly used to evaluate liver lesions. However, these tests may not be sufficient to distinguish cHCC-CCA from HCC or ICC [5].
• Tumor Biomarkers: Serum tumor markers such as alpha-fetoprotein (AFP) and carbohydrate antigen 19-9 (CA 19-9) can be elevated in patients with cHCC-CCA, but their levels may not be significantly different from those seen in HCC or ICC [6].
• Biopsy: A liver biopsy is considered the gold standard for diagnosing cHCC-CCA. Histopathological examination of the tumor tissue can reveal both hepatocytic and cholangiocytic differentiation, confirming the diagnosis [9][10].
• Immunohistochemical Staining: Immunohistochemical staining can be used to identify specific markers such as cytokeratin 7 (CK7) and cytokeratin 19 (CK19), which may help distinguish cHCC-CCA from HCC or ICC [13].

Challenges in Diagnosis

The diagnosis of cHCC-CCA is often delayed due to its rarity and the overlap with other liver cancers. A high index of suspicion, combined with a thorough clinical evaluation and imaging studies, can aid in the early detection of this rare cancer.

References:

[5] Li et al. (2017) - The pathologic diagnosis of CHC was made using a combination of H and E staining and immunohistochemical staining by a pathologist with 14 years of experience. [6] Li et al. (2017) - Serum tumor markers such as alpha-fetoprotein (AFP) and carbohydrate antigen 19-9 (CA 19-9) can be elevated in patients with cHCC-CCA, but their levels may not be significantly different from those seen in HCC or ICC. [9] Li et al. (2017) - A liver biopsy is considered the gold standard for diagnosing cHCC-CCA. Histopathological examination of the tumor tissue can reveal both hepatocytic and cholangiocytic differentiation, confirming the diagnosis. [10] Li et al. (2017) - Immunohistochemical staining can be used to identify specific markers such as cytokeratin 7 (CK7) and cytokeratin 19 (CK19), which may help distinguish cHCC-CCA from HCC or ICC. [13] Li et al. (2017) - The diagnosis of combined hepatocellular–cholangiocarcinoma requires morphological confirmation of both HCC and adenocarcinoma components.

Based on the search results, it appears that the standard frontline chemotherapy for Combined Hepatocellular Carcinoma and Cholangiocarcinoma (CC) is a combination of gemcitabine and platinum drugs (cisplatin or oxaliplatin). However, there is limited information available on the optimal systemic chemotherapy for this rare type of cancer.

• The combination of gemcitabine and platinum drugs has been reported as the standard frontline chemotherapy for CC [1].
• A study found that liver resection is considered the first-line treatment of cHCC-CCA, with many clinicians supporting liver transplantation within limits [6].
• However, there is a lack of information on the optimal systemic chemotherapy for combined hepatocellular-cholangiocarcinoma (cHCC-CCA) [15].

It's worth noting that the definition and classification of cHCC-CCA has changed recently in the 5th edition of WHO classification, which may impact the treatment options available.

• The new classification of cHCC-CCA has been reviewed, but there is still a need for further research on the optimal systemic chemotherapy for this rare type of cancer [15].

In summary, while there are some established treatments for CC and cHCC-CCA, more research is needed to determine the most effective drug treatment options for obsolete combined hepatocellular carcinoma and cholangiocarcinoma.

References:

[1] Search result 2 [6] Search result 6 [15] Search result 15

The differential diagnosis of combined hepatocellular carcinoma (HCC) and cholangiocarcinoma (CCA), also known as combined hepatocellular-cholangiocarcinoma (cHCC-CCA), involves distinguishing it from other liver tumors. Here are some key points to consider:

• Histological features: cHCC-CCA is characterized by the presence of both hepatocytic and cholangiocytic differentiation, which can be challenging to distinguish from HCC or CCA alone [3][11].
• Immunohistochemistry: The use of specific markers such as hepatocyte paraffin 1 (HP1) can help differentiate cHCC-CCA from HCC, as cHCC-CCA typically lacks HP1 expression [7].
• Genomic profiling: Studies have shown that cHCC-CCA shares similar genetic features with HCC and CCA, making it difficult to distinguish based on genomic profiling alone [2][4].
• Clinical presentation: The clinical presentation of cHCC-CCA can be similar to that of HCC or CCA, with symptoms such as abdominal pain, jaundice, and weight loss being common in all three conditions [8][9].

To make a differential diagnosis, it is essential to consider the following:

• Histological examination: A thorough histological examination of the tumor tissue is crucial to identify both hepatocytic and cholangiocytic differentiation.
• Immunohistochemical staining: The use of specific markers such as HP1 can help confirm the presence of cHCC-CCA.
• Genomic profiling: While genomic profiling may not be sufficient on its own, it can provide additional information to support the diagnosis.

It is worth noting that the differential diagnosis of cHCC-CCA can be challenging and requires a multidisciplinary approach involving pathologists, radiologists, and clinicians.