Current Evidence Tumors of the hepatobiliary tree in children are more likely to be metastases rather than primary tumors of the liver ( Table 98.6 ) Of the primary tumors, hepatoblastoma (HB) and hepatocellular carcinoma (HCC) are the most common ( Fig 98.2 ) HB usually occurs in patients younger than years and is associated with risk factors such as overgrowth syndromes (e.g., Beckwith–Wiedemann), prematurity, and familial adenomatous polyposis (FAP) In contrast, HCC is more common in patients older than years, particularly in children older than 10 years Risk factors include chronic liver injury from inborn errors of metabolism such as tyrosinemia, glycogen storage disease type I, chronic hepatitis, chronic iatrogenic androgen exposure, or cirrhosis for any reason In general, these latter risk factors rarely lead to cancer in childhood FIGURE 98.2 A: Wilms tumor Computed tomography (CT) scan of the abdomen reveals a large mass entirely replacing the left kidney B: Hepatoblastoma CT scan of the abdomen reveals a large mass arising from the inferior aspect of the liver The mass enhances heterogeneously with multiple low-density foci C: Neuroblastoma CT scan of the abdomen reveals a large mass originating from above the right kidney The mass crosses the midline and displaces the right kidney laterally and inferiorly The right adrenal gland is not visualized Clinical Considerations Clinical Recognition Typical presenting symptoms include abdominal mass, abdominal pain, and, very rarely, an acute abdomen from massive hepatomegaly or tumor rupture Other primary liver masses include embryonal sarcoma (extremely rare), nonmalignant vascular lesions such as infantile hemangioendothelioma, and malformations such as hamartoma Focal nodular hyperplasia can cause a mass on liver imaging but rarely has associated symptoms or hepatic enlargement Metastatic disease usually is characterized by diffuse nontender enlargement of the liver or multiple small nodules rather than a single dominant mass Neuroblastoma and advanced hematologic malignancies such as ALL, AML, lymphoblastic lymphoma, and Burkitt’s commonly metastasize to the liver Although tumors may block biliary drainage, hepatic synthetic function is rarely affected by malignancy in the liver Clinical Assessment Emergent management is rarely required except in the extremely rare setting of liver failure (see Chapter 91 Gastrointestinal Emergencies ), tumor rupture that may require rapid repletion of intravascular volume and blood loss, or severe coagulopathy (see Chapter 93 Hematologic Emergencies ) History may elicit risk factors or systemic symptoms such as malaise and anorexia that are more common with HCC than HB Pain does not help with the differential diagnosis but does require management Jaundice is most common with HCC but can occur with all liver tumors Management Initial workup should include measurement of AST, ALT, total and direct bilirubin, CBC, PT, PTT, and fibrinogen Alpha fetoprotein (AFP) can be elevated in both HB and HCC It is important to note that high AFP values are normal in the first months of life, especially in premature infants Initial diagnostic imaging should include an ultrasound, which can help identify if a palpable mass is likely to be hepatic in origin and if the liver contains one or multiple masses If a CT scan is done in the ED, it is important to give intravenous contrast to look for intravascular extension of tumor from the hepatic veins, into the inferior vena cava, and possibly into the right atrium Renal function should be checked before giving intravenous contrast Since HB can metastasize to the lungs, consider performing a chest CT scan at the same time in patients younger than 10 years who have a primary liver tumor Children who are clinically stable and have a new liver tumor may be discharged from the ED to the care of a pediatric surgeon experienced with liver tumors or a pediatric oncologist If the patient is unstable, they should be admitted to a center with experience in treating childhood malignancies Tumors of the Pancreas Pancreatic tumors in children are very rare They may develop in the setting of a predisposition, such as multiple endocrine neoplasias type (MEN-1) syndrome, which is associated with insulinomas of the pancreas Insulinomas will present with signs and symptoms of hypoglycemia and a history of “irrational behavior.” Other tumors of the pancreas cause either an abdominal mass or vague, nonspecific abdominal symptoms The differential diagnosis of a pancreatic mass includes nonmalignant adenoma or cystadenoma as well as malignant entities Malignant tumors of the pancreas in children may be cystadenocarcinoma, pancreatoblastoma, an embryonal tumor, or an endocrine tumor such as insulinoma, gastrinoma, or VIPoma ( Table 98.6 ) The pancreas may also be affected by metastatic disease from end-stage refractory cancer such as neuroblastoma or rhabdomyosarcoma A thorough history and physical examination should assess for endocrinologic ramifications that require medical management, such as hypoglycemia The evaluation should include a serum AFP, which can be elevated in pancreatoblastoma Diagnostic imaging may include a CT scan or MRI of the abdomen, but these tests are rarely needed in the ED The patient with a newly diagnosed pancreatic tumor may be discharged to home if the patient is otherwise well appearing and if arrangements have been made for an appropriate evaluation, including consultation with a pediatric surgeon, to continue in the outpatient setting If the patient is ill, or if appropriate follow-up is unclear, then it is safest to admit the patient to the hospital Surgical intervention is an important facet of the management plan for patients with pancreatic tumors, as several pancreatic tumors may be managed with surgery alone Tumors of the Gastrointestinal Tract Tumors in the gastrointestinal (GI) tract in children include lymphomas, leukemias, gastrointestinal stromal tumors (GISTs), LCH, desmoplastic small round cell tumor, and colorectal carcinomas ( Table 98.6 ) Risk factors for GI lymphomas include primary immunodeficiency Neurofibromatosis type increases the risk of GIST FAP, Li–Fraumeni syndrome, and ulcerative colitis increase the risk of colon cancer Common presentations include nonspecific symptoms such as weight loss, nausea/vomiting, loss of appetite, change in bowel habits, abdominal distention, or abdominal pain Chronic GI blood loss can cause iron-deficiency anemia Abdominal distention from masses or ascites may be present Severe GI bleeding is a rare presentation of GI malignancy but one that requires immediate management as in Chapter 33 Gastrointestinal Bleeding Symptoms of intermittent GI obstruction may be present Complete obstruction is an extremely rare presentation and may require urgent surgical intervention The lead point for intussusception in children older than years may be a primary GI lymphoma Incidental findings on appendectomies in children can include Burkitt lymphoma or carcinoid tumor Lymphomas involving the GI tract and/or mesenteric nodes in children include Burkitt lymphoma and large cell lymphoma ( Table 98.5 ) Advanced Burkitt lymphoma should be suspected in patients with a rapidly evolving clinical picture of progressive abdominal distention, abdominal masses, and/or ascites with associated elevation in uric acid, LDH, or renal insufficiency Colorectal carcinoma is ... have a new liver tumor may be discharged from the ED to the care of a pediatric surgeon experienced with liver tumors or a pediatric oncologist If the patient is unstable, they should be admitted... and if arrangements have been made for an appropriate evaluation, including consultation with a pediatric surgeon, to continue in the outpatient setting If the patient is ill, or if appropriate