Malignant primary hepatic tumors in children include lesions unique to the pediatric age group and others that are more common in adults. Important considerations when evaluating a child with a liver tumor are the age of the patient, laboratory findings, and specific imaging features. The most common primary malignant hepatic tumor in the pediatric population, hepatoblastoma occurs almost exclusively in patients younger than 5 years with no history of liver disease. Hepatoblastoma is associated with elevated serum α-fetoprotein (AFP) level and appears predominantly solid. Hepatocellular carcinoma (HCC) is the most common malignant liver tumor in older children, often with a history of liver disease. HCC is associated with elevated serum AFP level and also appears as a solid mass. Fibrolamellar carcinoma occurs in adolescents without elevated AFP level and contains a T2-hypointense fibrous scar that usually does not enhance. Undifferentiated (embryonal) sarcoma occurs in young children, contains cystic and mucoid components, and mimics a cyst at magnetic resonance imaging and computed tomography but appears solid at ultrasonography. Epithelioid hemangioendothelioma is a multifocal vascular tumor in older children with a distinctive imaging appearance of confluent peripheral nodules with adjacent capsular retraction. Angiosarcoma rarely occurs in young children and frequently shows evidence of hemorrhage. Embryonal rhabdomyosarcoma of the biliary tree is unique to children, usually under 5 years of age, and frequently demonstrates an intraductal growth pattern. Knowledge of the pathologic features of these tumors and their imaging appearances can help radiologists offer an appropriate differential diagnosis and management plan.
Pancreatic neoplasms are rare in children and have a different histologic spectrum and prognosis than those in adults. In general, these tumors are well demarcated with expansile rather than infiltrating growth patterns. They may be quite large at diagnosis, and central cystic necrosis is common. They infrequently cause biliary duct obstruction. The imaging appearance of each neoplasm reflects its pathologic features. Pancreatoblastoma is the most common pancreatic neoplasm in young children. At imaging, pancreatoblastomas are heterogeneous and often multilocular with hyperechoic and enhancing septa. Solid-pseudopapillary tumor occurs in adolescent girls. It is heterogeneous in internal architecture, with a mixture of solid and cystic hemorrhagic and necrotic elements. This tumor is distinguished by its fibrous capsule and hemorrhagic nature, which are best shown at magnetic resonance imaging as a dark rim on T1- or T2-weighted images and hyper-intense foci on T1-weighted images, respectively. Islet cell tumors in children are insulinomas or gastrinomas. These tumors manifest early due to hormonal syndromes and are distinguished by their small size, homogeneous appearance, and intense enhancement with intravenous contrast material. All pancreatic neoplasms in children are capable of producing metastases, usually to the liver and lymph nodes; however, on the whole, these tumors have a better clinical outcome than most pancreatic tumors in adults. Knowledge of the differential diagnosis of pancreatic masses in children and their relatively good prognosis may promote correct preoperative diagnosis and appropriate treatment.
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