Carcinoma of the Liver in Children and Adolescents

Abstract

Liver cancer, predominantly hepatocellular carcinoma, is the second most common cause of cancer-related death in adults. Although infrequent in children, hepatocellular carcinoma is a terrifying diagnosis. Rising levels of obesity and obesity-associated lipid metabolic reprogramming of hepatocytes are increasing the prevalence of lipid-rich hepatocellular carcinoma in young adults. Most pediatric liver cancers occur in otherwise healthy liver, with some exceptions such as progressive familial intrahepatic cholestasis, hereditary tyrosinemia, alpha-1-antitrypsin deficiency, and genetic hemochromatosis. In the last decade, although aggressive multidisciplinary treatments including surgical resection and chemotherapy have remarkably improved patient outcomes in terms of decreased recurrence rate and increased overall survival rate, in children with unresectable liver cancer, the 5-year survival rate is still less than 20%. This chapter provides an overview of malignant epithelial tumors of the liver in children and adolescents. Hepatocellular carcinoma, lipid-rich hepatocellular carcinoma, fibrolamellar carcinoma, and cholangiocellular carcinoma are discussed.

Pediatric Hepatocellular Adenomas: What Is Known and What Is New?

“…The majority of HCAs lack symptoms and are incidentally diagnosed [21,22]. Patients can also present with vague abdominal pain that appears to be non-positional, have no specific modifying factors, and can spontaneously increase or decrease in severity [22,23]. Other presentations can mimic acute appendicitis, malrotation, kidney stones, and gastroenteritis [21,22].…”

“…In contrast to adults, a significant subset of pediatric HCAs arise in the context of genetic disorders or other acquired conditions and their HCAs are more likely to be diagnosed incidentally during the surveillance imaging. Some asymptomatic cases are detected based upon suspicion by a caretaker or a family member [23]. In contrast, ruptured HCAs, can have a wide range of symptoms from vague abdominal pain to hypotensive crisis, peritonitis, and shock.…”

Chemokines in Primary Liver Cancer

“…Obtaining representative material during a biopsy allows for immunohistochemical and molecular diagnostics, which is of particular importance in the case of poorly differentiated cancer. Nevertheless, the use of a core needle biopsy carries a risk of bleeding, pain and the possibility of spreading cancer cells along the canal after the biopsy needle [ 66 ].…”

Liver Biopsy Handling of Metabolic-Associated Fatty Liver Disease (MAFLD): the Children’s Hospital of Eastern Ontario grossing protocol

“…The liver biopsy is a paramount procedure in internal medicine and gastroenterology, aiming to identify a broad spectrum of etiologies ranging from abnormal values of liver function tests to structural changes or injuries at the hepatocellular level. 1 – 5 Clinical evaluation entails a range from asymptomatic individuals to acute liver failure prompting liver transplantation. In the most recent definition of acute hepatitis, the American Association for the Study of Liver Disease categorizes an acute increase of liver biochemical values within six months of onset in a patient apparently not showing a pre-existing liver disease, while if the threshold of 6 months is over, the definition of chronic hepatitis is in place.…”