hepatic cancer

7/28/2019 Hepatic Cancer

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Cancer in Children

HEPATIC TUMOURS


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Introduction

Child presents with mass in the quadrant of theabdomen , the differential diagnosis includehepatic malignancy must be distinguished

from: benign hepatic tumor, non neoplastichepatomegali,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 ofhepatoblastoma 5-6 time higher thanhepatocellular carcinoma.


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HEPATOBLASTOMA Hepatoblastoma is the most common malignant

hepatic tumor in children, hepatocellularcarcinoma is more common due to endemichepatitis 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 Japanto be higher than Western.

Ratio males and female= 1,2 : 1

More than 80% tumor are seen in child 3 yearsage or younger and 45% of patient arediagnosed during first years of life.


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Genetic and biology

Hepatoblastoma may occur in association with

variety of - congenital anomalies,disorder, and

enviromental factors.

Beckwith-Wiedemann syndrome and hemihypertrophy , rhabdomyosarcoma, and

adrenocortical tumors an increase risk of thistumor.

Abnormalities of the short arm chromosome 11have 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 frequentlyobserved.

DNA content analyses have shown patient with

diploid have better prognosis than aneuploid


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Pathology

Hepatoblastoma is usually soliter and 20% oftumor extend multifocally or infiltrate diffuselyinto the liver.

Microscopically : pure epithelial hepatoblastomais distinguished from mixed hepatoblastoma,which contains mesenchymal tissue in additionto epithelial elements.

Epithelial component exhibit a range of

differentiation represented by fetal, embryonaland anaplastic cells.


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Diagnosis Anorexia; weight loss, anemia.

Alpha-fetoprotein (AFP) is raised above theupper limited more than 98% patient.

Thrombocytosis; normochromic normocytic

anemia is usually.

Hypercholesterolaemia or osteoporosis and latermay be complicated by pathological fracture.

Imaging study: plain radiography, USG; CT; MRI

Bone scan should be done to investigatemetastatic spread. Bone marrow aspirates areunnecessary.


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Staging

Pediatric Oncology Group USA/Children CancerGroup:

Stage I: tumor is define as a tumor resectedcompletely 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


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Treatment

1. Surgical treatment :

Complete surgical resection most effective treatment, butless than 50% tumor are resectable at time of diagnosis.

The tumor is deemed resectable if it is localized in one ortwo segment of the liver. Segmentectomy or standardlobectomy.

2. Chemotherapy ( see protocol)

Iphosphamide: 1.5/m2 for 3 days

Cisplatin 20mg/m2daily dose

Adriamycin 20 mg/m2


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Hepatocellular carcinoma Hepatocellular carcinoa accounts for 10-30% of

primary, malignant, hepatic tumors in Westernand Japan, but is more prevalent andoutnumbers hepatoblastoma in areas wherehepatitis B virus infection is endemic.

Tumor usually occurs in children older than 5years. Male dominant than female.

Hepatitis B virus infection is causally withdevelopment of hepatocellular carcinoma.

Tumor associated with number of underlyingdisease: tyrosinemia, biliary atresia, idiopaticneonatal hepatitis, alpha 1- trypsin deficiency


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Pathology

The gross and microscopic feature ofhepatocellular carcinoma is similar in childrenand adult, except for lower incidence of

underlying cirrhosis. Children 5-30% cirrhosis ispresent, in adult 50-85%.

Tumor as multiple nodulus or diffuse infiltratingmass involving both lobes of the liver.

The cut surface is often bile stained, andhemorrhagic and necrosis are more often thanhepatoblastoma.


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Diagnosis Sign: abdominal pain; a palpable mass

and hepatomegali is frequently.

Fever, weight loss and jaundiceoccasionally observed. Shock and intraabdominal bleeding may be initialmanifestation of tumor rupture.

Anemia and hiperbilirubinemia, serumAFP 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)


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Treatment

Complete resection is the basis of successfultreatment, but resectability of the tumor is 10-20%. 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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hepatic cancer

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