Preface

The liver is the major organ in which our body's metabolic activities are carried out. Food is digested in the gastrointestinal tract and absorbed by the small intestines. These absorbed foods come to the liver through the venous circulation of the small intestines. The liver takes these foods through the portal vein and transforms them into a shape that the body can use, and delivers them to the vena cava and from there to the systemic circulation via hepatic veins. This circulation between the intestines and the liver is called Hepatic Portal Circulation.

The vena porta is the main venous structure that brings blood flow from the intestines to the liver. The vena porta is formed by the union of three venous structures. These structures are splenic vein, vena mesenterica superior, and vena mesenterica inferior.

This portal circulation is an important way to carry the absorbed foods to the liver, as well as for infections and cancer to metastasize by the venous spread. In appendicitis, the infection reaches the liver, causing pylephlebitis. In bowel cancer, cancer cells entering the mesenterica inferior vein or mesenterica superior vein go towards the liver due to portal venous flow, therefore the place where colon cancer most metastasizes is the liver. When these metastatic masses reach a certain size, they enter the venous circulation of the liver, from here to the vena cava and from there to the right ventricle. The blood entering the right ventricle goes from here to the lungs via the pulmonary artery. Cancer cells are retained in the lung microcirculation and metastatic masses form in the lung.

Metabolic events in the liver increase the temperature of the liver. The liver is a warmer organ than normal body temperature. The heat that occurs as a result of metabolic events is distributed throughout the body due to blood circulation.

Some organs in our body are double and some organs are single. For example, there are two kidneys in the body. The liver is a single. An amazing feature of the liver is that each tissue part of the liver performs the same function. In other words, if a part of the liver is removed, the other parts of the liver do the same job, so there will be no significant change in function. In order for the remaining liver to function properly after liver resection, the remaining liver (FLR) must be of sufficient volume.

There are many diseases of the liver. Metabolic diseases, neoplastic diseases, hepatopathy due to cardiac pathologies, infective diseases such as Hepatitis E, cirrhosis, etc. are present. These diseases can lead to impaired liver function and cause metabolic complications in the human body.

Neoplastic diseases can be benign or malignant. Malignant diseases can be primary or secondary. The most common secondary malignant neoplastic disease is metastatic cancer caused by colorectal cancer. Diagnosis can be made by radiological imaging. These methods include computerised tomography and MRI. A percutaneous liver biopsy may be required for diagnosis. Surgical metastasectomy or regular

resection can be performed in the treatment. Interventional radiological procedures are another option used in the treatment of liver tumors. Chemotherapy has an important place in the treatment of liver tumors.

Various diseases of the liver that require surgical and medical treatment are examined in our book. Our authors wrote their subjects by combining their knowledge with their experiences.

Have a pleasant reading experience.

**Omer Engin** Izmir Tepecik Eğitim ve Araştırma Hastanesi, Turkey

> **Vijay Gayam** Interfaith Medical Center, United States of America

**Chapter 1**

**Abstract**

and treatment of hepatoblastoma.

resection, liver transplantation

improved in recent years [6].

**2. Diagnosis**

**1. Introduction**

Diagnosis and Treatment of

Hepatoblastoma: An Update

*Chengzhan Zhu, Bingzi Dong, Xin Chen and Qian Dong*

Hepatoblastoma is a rare but the most common solid tumor in children. The incidence is gradually increasing. The international collaboration among four centers in the world has greatly improved the prognosis of hepatoblastoma. They formed the Children's Hepatic Tumor International Collaboration (CHIC) to standardize the staging system (2017 PRETEXT system) and the risk factors for tumor stratification. Multimodal therapy has become the standard for the management of hepatoblastoma, including surgical resection, liver transplantation, chemotherapy, and so on. Surgery is the primary treatment of early stage hepatoblastoma. Threedimensional reconstruction is helpful for preoperative evaluation of large tumors, assisting extended hepatectomy for patients in PRETEXT III or IV. Neoadjuvant therapy is useful for reducing the tumor volume and increasing the resectability. Primary liver transplantation is recommended for advanced hepatoblastoma. The lungs are the most common metastatic organ, the treatment of which is critical for the patient's long-term survival. We reviewed the recent progress in the diagnosis

**Keywords:** hepatoblastoma, PRETEXT, stratification, neoadjuvant, surgical

Hepatoblastoma is the third most commonly diagnosed intra-abdominal solid tumor [1]. It is also the most common primary hepatic malignancy in children [2]. More than 90% of hepatoblastoma occur in children under the age of 5 years [3, 4]. Although its absolute incidence is very low, its growth rate is gradually increasing, which increased from 1.89 per 1,000,000 in 2000 to 2.16 per 1,000,000 in 2015, with an annual percentage change of 2.2%. This increase mainly occurs in male children between 2 and 4 years of age, which was found to be an independent predictor for short overall survival [5]. With the development of multimodal treatment and cooperation between international organizations, the prognoses have been greatly

Clinical manifestations are not typical at the early stage of hepatoblastoma. There would be epigastric or total abdominal distention, nausea, vomit, loss of appetite, abdominal pain, diarrhea, jaundice, even varicosity of abdominal wall,

#### **Chapter 1**
