Clinico-Pathological study of Paediatric Liver Tumors.

Ravindar, A (2010) Clinico-Pathological study of Paediatric Liver Tumors. Masters thesis, Madras Medical College, Chennai.

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Abstract

INTRODUCTION : Primary neoplasms of the liver occur rarely during childhood and constitute only 0.3-2% of all pediatric tumors. However, they comprise a variety of entities including benign and malignant epithelial, as well as mesenchymal tumors, the most common of these being hepatoblastoma. Other malignant liver tumors are quite rare and include biliary rhabdomyosarcoma, angiosarcoma, rhabdoid tumor, and undifferentiated sarcoma. The commonly seen benign liver tumors in children are infantile hemangioma, mesenchymal hamartoma, and focal nodular hyperplasia. Rare benign tumors are hepatic adenoma and teratoma. Clinical presentation, especially in young children is relatively uniform with abdominal enlargement and a painless tumor, and often specific symptoms develop late. Prerequisites for clinical diagnosis are a comprehensive laboratory workup and good quality imaging mainly with ultrasound, as well as CT and/or MRI scans. Histological diagnosis is essential for differential diagnosis. Major progress has been achieved during the last decades in the treatment of malignant liver tumors in children, both in chemotherapy and in surgical management. Surgery is the mainstay of treatment for all benign and malignant liver tumors. Prognosis nowadays usually is good in all benign tumors and hepatoblastoma, as well as in some other rare malignancies, but dismal in hepatocellular carcinoma and other chemotherapy non sensitive malignant tumors. The future in the treatment of children with advanced malignant liver tumors lies in primary liver transplantation. AIM : 1. To study the incidence and age distribution of various liver tumors in children. 2. To study the mode of presentation and clinical profile of both benign and malignant liver tumors. 3. To study the role of tumor markers and imaging techniques in the preoperative workup of these tumors. 4. To study the role of primary liver resections in achieving disease free survival. 5. To assess the effectiveness of adjuvant and neoadjuvant chemotherapy in downstaging the disease. 6. To study the outcome based on morbidity and mortality profile of the children treated within the period of study. 7. To identify pitfalls (if any) in the management protocol to improve the long term survival. 8. To suggest guidelines for future management. MATERIALS AND METHODS : This study on liver tumors in children is based on 34 patients admitted to the Institute of Child Health and hospital for children [ICH & HC], Egmore during the period from January 2005 to December 2009. The study includes 24 boys and 10 girls between the age group 6 days to 9 years. Data collected for each patient had been recorded in the enclosed chart. CONCLUSION : Surgery of liver has a special point in paediatric surgical practice because Hepatic tumors are major disorders affecting children with high morbidity and mortality rates. This study covers 34 patients over a 5 year period and compares well with other established centers in India and around the world. The results are not as abysmal as previously thought of. Experience of the surgeon, familiarity to complex anatomy, strict adherence to anatomical planes, advances in anaesthesia, surgical technique, use of newer surgical tools (like CUSA, Intraoperative ultrasound, staplers etc) and chemotherapy have led to a significant improvement in the prognosis of children with hepatoblastoma. Outcomes for hepatocellular carcinoma remain poor. Anatomical resection becomes more comfortable in malignant tumors which are initially treated by preoperative chemotherapy. Liver transplantation is useful in patients with unresectable tumors. Long term goal of the centers undertaking major hepatic resections for liver malignancies in children must be pre tuned to liver transplantation, which will become the gold standard in the management of liver tumors in children in the future. Continued cooperation of multiinstitutional pediatric cancer study groups will be required to achieve additional advances in the treatment of malignant liver neoplasms.

Item Type: Thesis (Masters)
Uncontrolled Keywords: paediatric liver tumors ; Clinico-pathological study.
Subjects: MEDICAL > Paediatric Surgery
Depositing User: Kambaraman B
Date Deposited: 28 Jul 2017 03:00
Last Modified: 28 Jul 2017 03:00
URI: http://repository-tnmgrmu.ac.in/id/eprint/2291

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