HEPATIC TUMORS
Hepatic malignancies in children usually indicate secondary metastasis. Primary liver tumors are rare in this age group, commonest being hepatoblastoma. Second commonest liver cancer, hepatocellular carcinoma is usually seen in adolescents and adults.
Uncommon liver tumors include malignant sarcoma or germ cell tumors and benign hamartoma, hemangioma and teratoma.Hepatoblastoma is an embryonal tumor of hepatic epithelial parenchyma, mostly seen in young children lt;3 years of age and occasionally associated with other congenital anomalies, e.g. hemihypertrophy, Beckwith- Wiedemann syndrome, diaphragmatic hernia, etc.
Clinically, hepatoblastoma usually present with asymptomatic abdominal mass with/without pain, fever, weight loss and vomiting. Rarely, isosexual precocious puberty or severe osteoporosis with pathologic fractures may be prominent feature due to altered steroid metabolism.
Diagnosis depends on abdominal CT-scan, biopsy and elevated #945;-fetoprotein levels. X-ray abdomen may reveal intra-tumor calcification in ~30% cases. Chest radiograph/CT is necessary to detect pulmonary metastasis.
Treatment: Complete surgical resection is the only realistic chance of long-term disease free survival in children with hepatoblastoma, though lt;50% cases have resectable tumors at the time of diagnosis.
However, pre-operative chemotherapy with Cisplatin alone or with Doxorubicin (PLADO) allows resection of initial unresectable tumor in most cases. Pulmonary metastasis needs surgery and chemotherapy.
Prognosis: Three-year survival is gt;90% in initially resected tumors, ~65% in initially unresectable tumors and ~10-20% in those with metastatic disease.
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