Pediatric Hepatoblastoma Follow-up

Updated: Sep 15, 2017
  • Author: Jennifer Reikes Willert, MD; Chief Editor: Max J Coppes, MD, PhD, MBA  more...
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Follow-up

Further Outpatient Care

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  • 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.

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Further Inpatient Care

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  • 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.

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Inpatient & Outpatient Medications

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  • 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.

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Transfer

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  • 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.

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Deterrence/Prevention

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  • 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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Complications

Tumor rupture may occur at diagnosis, resulting in acute abdomen or severe hemorrhage, both of which constitute medical emergencies. Intraoperative and postoperative complications may occur as a result of resection or biopsy procedures.

Complications can develop with the administration of chemotherapy. Myelosuppression and immunosuppression place the patient at risk for bleeding and infection. After several cycles of therapy, organ toxicity may occur; for example, renal function or hearing may be impaired.

Posttransplantion complications can develop and require close long-term follow-up by the liver transplant team.

Particular attention must be paid to cardiac, renal, and hearing status to assess for the long-term toxic effects of anthracyclines, cisplatin, or carboplatin. One of the most important adverse effects of platinum chemotherapy is hearing loss. A Cochrane Database review of 3 studies that evaluated the use of the chemoprotective agent amifostine versus no additional treatment did not come to any definitive conclusions. Further research is needed regarding the usefulness of possible drugs to prevent hearing loss in children treated with platinum chemotherapy. [46]

Psychosocial effects of frequent painful procedures, hospitalizations, and interference with normal childhood growth and development must be addressed, and children and families must be referred to appropriate specialists when needed. The family's psychosocial needs are affected greatly by having a child with cancer.

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Patient Education

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  • Medications: To ensure compliance and good medical care, patient and family understanding regarding the importance of treatment and the toxic effects of the medications is critical. In addition, patients and their families should learn to recognize and identify signs and symptoms of complications that require urgent medical care.

  • Long-term follow-up surveillance: After completion of therapy, patients in whom treatment was successful require close surveillance for any signs or symptoms of recurrent disease. Follow-up care includes monitoring AFP levels, physical examination, and diagnostic imaging. Because most recurrences occur during the first 2 years following treatment, most protocols recommend close follow-up monitoring during this interval. Hepatoblastoma does not usually recur more than 3 years after completion of therapy.

  • Long-term issues: Growth and development and long-term toxic effects on organs are long-term issues. Patients who remain free of recurrent disease for 5 years are considered cured; long-term follow-up monitoring to assess the impact of therapy on growth, development, and organ toxicity is essential. Patients are usually monitored by pediatric oncologists, but some sequelae may require the involvement of other subspecialist health care providers.

  • Other issues: Most centers have late effects clinics, and all children treated for cancer should continue to see their oncology providers regularly to monitor for potential long-term complications of therapy. When appropriate, most centers help transition to an adult provider, with guidelines on what to watch for and which tests should be performed to monitor for potential late effects. A cancer-preventive lifestyle is encouraged and includes avoiding passive or primary tobacco exposure, wearing sunscreen, healthy eating habits, maintaining a healthy weight, and an exercise regimen.

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