HEPATIC TUMOURS

Cancer in Children

Introduction

Child presents with mass in the quadrant of the abdomen , the differential diagnosis include hepatic malignancy must be distinguished from: benign hepatic tumor, non neoplastic hepatomegali, neuroblastoma, nephroblastoma. Two-third of hepatic neoplasm were malignant (50-60%) and approximately 90% were: epithelial tumors, hepatoblastoma or hepatocellular carcinoma. Ratio hepatoblastoma to hepato cellular carcinoma is 1,8 :1 and incidence of hepatoblastoma 5-6 time higher than hepatocellular carcinoma.

HEPATOBLASTOMA

Hepatoblastoma is the most common malignant hepatic tumor in children, hepatocellular carcinoma is more common due to endemic hepatitis B infection with its vertical transmission. The incidence in children under 15 years is less than 1 per 1.000.000 in North America, in Japan to be higher than Western. Ratio males and female= 1,2 : 1 More than 80% tumor are seen in child 3 years age or younger and 45% of patient are diagnosed during first years of life.

Genetic and biology

Hepatoblastoma may occur in association with variety of congenital anomalies,disorder, and enviromental factors. Beckwith-Wiedemann syndrome and hemi hypertrophy , rhabdomyosarcoma, and adrenocortical tumors an increase risk of this tumor. Abnormalities of the short arm chromosome 11 have been shown and loss of heterozygosity of 11 p15 has frequently been observed. Cytogenetic of tumor cell have shown that trisomy of chromosome 2 and 20 is frequently observed. DNA content analyses have shown patient with diploid have better prognosis than aneuploid

Pathology

Hepatoblastoma is usually soliter and 20% of tumor extend multifocally or infiltrate diffusely into the liver. Microscopically : pure epithelial hepatoblastoma is distinguished from mixed hepatoblastoma, which contains mesenchymal tissue in addition to epithelial elements. Epithelial component exhibit a range of differentiation represented by fetal, embryonal and anaplastic cells.

Diagnosis
Anorexia; weight loss, anemia. Alpha-fetoprotein (AFP) is raised above the upper limited more than 98% patient. Thrombocytosis; normochromic normocytic anemia is usually. Hypercholesterolaemia or osteoporosis and later may be complicated by pathological fracture. Imaging study: plain radiography, USG; CT; MRI Bone scan should be done to investigate metastatic spread. Bone marrow aspirates are unnecessary.

Staging
Pediatric Oncology Group USA/Children Cancer Group: Stage I: tumor is define as a tumor resected completely at initial laparatomy. Stage II: as a tumor with microscopic residue Stage III: as a tumor with gross residual tumor. Stage IV: as a distant matastases

Treatment

1. Surgical treatment : Complete surgical resection most effective treatment, but less than 50% tumor are resectable at time of diagnosis. The tumor is deemed resectable if it is localized in one or two segment of the liver. Segmentectomy or standard lobectomy. 2. Chemotherapy ( see protocol) Iphosphamide: 1.5/m2 for 3 days Cisplatin 20mg/m2daily dose Adriamycin 20 mg/m2

Hepatocellular carcinoma

Hepatocellular carcinoa accounts for 10-30% of primary, malignant, hepatic tumors in Western and Japan, but is more prevalent and outnumbers hepatoblastoma in areas where hepatitis B virus infection is endemic. Tumor usually occurs in children older than 5 years. Male dominant than female. Hepatitis B virus infection is causally with development of hepatocellular carcinoma. Tumor associated with number of underlying disease: tyrosinemia, biliary atresia, idiopatic neonatal hepatitis, alpha 1- trypsin deficiency

Pathology

The gross and microscopic feature of hepatocellular carcinoma is similar in children and adult, except for lower incidence of underlying cirrhosis. Children 5-30% cirrhosis is present, in adult 50-85%. Tumor as multiple nodulus or diffuse infiltrating mass involving both lobes of the liver. The cut surface is often bile stained, and hemorrhagic and necrosis are more often than hepatoblastoma.

Diagnosis

Sign: abdominal pain; a palpable mass and hepatomegali is frequently. Fever, weight loss and jaundice occasionally observed. Shock and intra abdominal bleeding may be initial manifestation of tumor rupture. Anemia and hiperbilirubinemia, serum AFP is high in 50-80% of patients. Possible presence of hepatitis B infection Imaging study: USG,CT,MRI Metastases spread is to lungs, regional lymph nodes and bone (rare)

Treatment
Complete resection is the basis of successful treatment, but resectability of the tumor is 1020%. Most children die within 12 months of diagnosis. Intensive chemotherapy : the result so far have generally been dismal. Combination of : iphosphamid ; cisplatin and adriamycin. Total hepatectomy and orthotopic liver transplantation appears to be effective.

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