November 27, 2022

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Fibrolaminous hepatocellular carcinoma:  A rare type of hepatocellular carcinoma!

Fibrolaminous hepatocellular carcinoma:  A rare type of hepatocellular carcinoma!

 

 

 

Fibrolaminous hepatocellular carcinoma:  A rare type of hepatocellular carcinoma! Patients with too large tumors or too many underlying diseases that cannot tolerate surgery can choose local treatment. Clinically, hepatic artery embolization and drug intervention are commonly used to shrink tumors, reduce complications, and prolong survival.


Fibrolamellar hepatocellular carcinoma (FL-HCC) mainly occurs in young children, adolescents and young adults. As there is no good diagnostic test method, there are no accurate data on its epidemiology. Currently, the only method of diagnosis is differential diagnosis by a pathologist through biopsy.

 

Unfortunately, clinically, when a teenager complains of prolonged abdominal pain or stomach pain, it is usually considered as stress or dietary problems such as lactose intolerance, and cancer is rarely considered. Therefore, the patient’s condition may be delayed for many years.

 

So, what are the characteristics of FL-HCC?

 

 

 



Features of FL-HCC


It is a rare and special type of hepatocellular carcinoma, accounting for only 1%-2% of the total incidence.

 

The vast majority of patients have no basis for liver cirrhosis, HB infection is rare, and AFP is mostly negative.

 

It is more common in the left lobe. It is often a single lobular lesion with a hard texture and swelling growth. It is clearly separated from normal liver tissue, and may have a capsule, which is bulky, usually larger than 10cm in diameter.

 

The stellate fibrous scar in the center of the tumor radiates to the surrounding and separates the tumor is an important feature. Another feature is that the center of the scar may have spotty calcification.

 

The lesion is often solitary, and some cases have small satellite lesions around the main lesion. Dilated bile ducts are rare, and calcification can be seen inside.

 

Under the microscope, the cancer cells are pleomorphic and contain strong eosinophilic cytoplasm. The cancer cells are arranged in nests, and a large number of lamellar fibrous tissues arranged in parallel appear between the nests.

 

It can happen at any midday age, but it happens better in young people. Among the primary liver cancers under the age of 35 without cirrhosis, FL-HCC accounted for 43%, and there was no significant gender difference.

 

 


Laboratory tests such as HBsAg negative, AFP, CEA, AKP, etc. are normal or slightly elevated.

 

Although FL-HCC has a large mass, it has a high surgical resection rate and a good prognosis.

 

CT plain scan showed low-density areas with sharp edges, internal cord-like structures and necrotic areas.

 

It is characterized by the appearance of calcification in the lesion. The calcification is mostly punctate or small round, with high density and located inside the lesion. Small satellite foci around the lesion can also be found at the same time.

 

The enhanced blood supply of the tumor parenchymal part is rich, and it shows early enhancement in the arterial phase, while the fibrous septum is relatively low-density. There is no enhancement in the central scar area in the delayed scan, and the display is clearer.

 

The central scar is in the form of a star-shaped or irregular lower-density shadow with clear borders, and spot-like calcification can be seen. The parenchyma of the arterial phase tumor is uniform or diffuse early enhancement, and the portal phase tumor parenchymal enhancement is reduced, and the density is slightly higher or lower than that of the surrounding liver tissue.

 

In the late stage, there may be portal vein invasion and hilar lymph node metastasis. The rate of hilar lymph node metastasis is higher than that of ordinary hepatocellular carcinoma.

 

 

 

 




Treatment of FL-HCC


In terms of treatment, if FL-HCC is confined to the liver, surgery can be selected to remove it. It is worth noting that FL-HCC is different from other liver cancers. Most patients do not have liver damage and cirrhosis. Therefore, after the tumor is surgically removed, the liver has a strong regenerative capacity and the patient can return to normal.

 

However, because early symptoms are easily overlooked, they are usually diagnosed when the tumor has metastasized to the outside of the liver and other locations. There is currently no effective treatment for this condition.

 

Patients with too large tumors or too many underlying diseases that cannot tolerate surgery can choose local treatment. Clinically, hepatic artery embolization and drug intervention are commonly used to shrink tumors, reduce complications, and prolong survival.

 

(source:internet, reference only)


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