Further Outpatient Care
Patients are periodically monitored in the clinic after each course of therapy to assess for complications and response to therapy. Myelosuppression and pancytopenia are common complications, and a CBC count with a platelet count is obtained once or twice weekly.
Some drugs, such as cisplatin and carboplatin, affect renal function and require close monitoring of electrolytes and oral or parenteral electrolyte supplementation.
Blood product support is provided when the hemoglobin drops below 8 g/dL, symptoms of anemia are present, the platelet count drops below 10,000 X 109/L, or any signs of bleeding are evident.
Fever must be treated as a medical emergency during therapy because the risk of a bacterial or fungal infection is high in patients with myelosuppression.
Children with central lines are susceptible to bacteremia and life-threatening sepsis. In addition, all children with central lines must receive appropriate antimicrobial prophylaxis against subacute bacterial endocarditis (SBE) for all procedures, including dental procedures.
Close contact with the liver transplant team is required for patients who require this treatment. All medical decisions for patients with this complex condition should be communicated to all members of the team including oncologists, primary surgeon, hepatologists, and transplant surgeons.
Late effects clinics are available at most major oncology centers, and children with hepatoblastoma should be referred to these clinics if they remain disease free for more than 2 years. Even if the risk of recurrence decreases with time, these children are still at risk for late effects, which include secondary cancers (etoposide and anthracycline), cardiotoxicity (anthracycline), renal toxicity (platinum agents), ototoxicity (platinum agents), and potential speech and developmental delays due to therapy administered.
Psychosocial team members, child life experts, medical social workers, nutritionists, and all care providers can help families adjust to life after cancer and can also help encourage a cancer preventive lifestyle for these at-risk patients.
Further Inpatient Care
Follow-up care
Children may be admitted to the hospital to expedite the diagnostic workup or when severe signs or symptoms are present. For medically stable patients, the workup can be performed in the outpatient setting. A central line is typically placed when the patient is scheduled for biopsy or resection. Double-lumen central lines are preferred if vessel access is adequate because this allows concurrent administration of multiple parenteral medications.
Multidisciplinary evaluation
The child is initially evaluated by a pediatric oncologist and surgeons with expertise in childhood malignancies. Evaluation should be performed at a pediatric cancer center. Once the diagnosis is established and the staging workup is completed, the patient and family are instructed on the diagnosis and therapeutic options. Most children and families are offered participation in cooperative group trials. Once the treatment plan is developed, chemotherapy is most frequently administered in the inpatient setting. However, with improvements in supportive care, some chemotherapy may be administered in the outpatient setting. Following completion of the treatment cycle, patients are discharged home with detailed instructions for home care and outpatient follow-up visits.
Liver transplantation
Patients who undergo liver transplantation require a multidisciplinary team with experienced hepatologist and liver transplant surgeons as well as the team outlined above.
Inpatient & Outpatient Medications
Infection prophylaxis
Chemotherapy agents cause myelosuppression and immunosuppression. Prophylaxis against Pneumocystis jiroveci, which causes Pneumocystis carinii pneumonia, is recommended for all patients. The drug of choice is trimethoprim-sulfamethoxazole (2.5 mg/kg/dose of trimethoprim administered orally twice daily) administered on 3 consecutive days per week. Prophylaxis is initiated before chemotherapy and is continued for at least 3 months after completing therapy.
Colony-stimulating factors
Granulocyte colony-stimulating factor (G-CSF) support has become common in pediatric oncology as the intensity of chemotherapy has increased. The doses recommended are 5-10 mcg/kg/d, starting 24-36 hours after the last dose of chemotherapy. G-CSF administration is continued for 10-14 days or until the absolute neutrophil count (ANC) is greater than 2,000-10,000/mcL.
Erythropoietin
The use of erythropoietin is discouraged because of reports that hepatoblastoma has receptors for this agent and may therefore be stimulated to grow from exogenous sources.
Transfer
With supervision by the oncology team, routine care can be performed by the primary care provider for patient convenience. CBC counts and blood chemistries may be obtained and blood products may be administered by primary care providers.
Some patients may even be evaluated and treated for febrile neutropenia by the primary care provider. However, the primary care provider must maintain close contact with the subspecialist physicians and transfer the patient to the pediatric oncology center for any complications that require specialized care.
Deterrence/Prevention
The cause of hepatoblastoma is unknown. Because onset of hepatoblastoma is in patients at a young age, investigators have focused on events before conception and during gestation. Factors for which evidence is limited or inconsistent include medications, hormones, birth characteristics, congenital anomalies, previous spontaneous abortion or fetal death, alcohol consumption, tobacco use, and paternal occupational exposures.
Children with hemihypertrophy or Beckwith-Wiedemann syndrome (BWS) and children born to individuals affected by familial adenomatous polyposis (FAP) should be screened regularly using blood α -fetoprotein (AFP) levels as dictated in current protocols. Children found to harbor a FAP mutation should be periodically monitored for the development of polyps by a gastroenterologist as they reach the teenage years.
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Clear cell hepatoblastoma. Hematoxylin and eosin stain. Image courtesy of Denise Malicki, MD.
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Embryonal hepatoblastoma. Hematoxylin and eosin stain. Image courtesy of Denise Malicki, MD.
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Fetal components of hepatoblastoma. Hematoxylin and eosin stain. Image courtesy of Denise Malicki, MD.
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Hepatoblastoma. Normal liver tissue. Hematoxylin and eosin stain. Image courtesy of Denise Malicki, MD.
