Clinico-Pathological study of Paediatric Liver Tumors.

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 ...