Pediatric Neuroblastoma Follow-up

Updated: Oct 09, 2017
  • Author: Norman J Lacayo, MD; Chief Editor: Max J Coppes, MD, PhD, MBA  more...
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Follow-up

Further Outpatient Care

The following are aspects of further outpatient care in patients with neuroblastoma:

  • Patients are periodically monitored in the clinic after each course of therapy to monitor for complications and to assess response to therapy with diagnostic imaging. Myelosuppression and pancytopenia are common complications, and a CBC count with platelet count is obtained as often as twice per week. Some drugs (eg, cisplatin, carboplatin, ifosfamide) affect renal function; thus, close monitoring of electrolytes is required, with oral electrolyte supplementation when necessary. Blood product support is provided when the hemoglobin drops to less than 8 g/dL, the platelet count drops to less than 10,000, or any signs of bleeding are present.
  • After completion of therapy, successfully treated patients require follow-up care and close surveillance for any signs or symptoms of recurrent disease. Follow-up care includes monitoring of urinary catecholamines, physical examination, and diagnostic imaging. Because most recurrences occur during the first 2 years following treatment, most protocols recommend close follow-up care during this interval.
  • Patients who remain free of recurrent disease for 5 years are considered cured, although rare late relapses have been reported. Long-term follow-up care to assess impact of therapy on growth, development, and organ toxicity is essential.
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Further Inpatient Care

The following are aspects of further inpatient care in patients with neuroblastoma:

  • Children with neuroblastoma are admitted to the hospital to expedite the diagnostic workup when unstable or significantly symptomatic.
  • In an asymptomatic child, workup can be performed in the outpatient setting.
  • A central line is commonly placed when biopsy or resection is scheduled in intermediate- or high-risk patients.
  • A pediatric oncologist and surgeons with expertise in managing childhood malignancies perform the initial evaluation.
  • Other subspecialists, such as neurosurgeons or radiation oncologists, may participate in patient care, especially in cases of cord compression.
  • Once the diagnosis is established and the staging workup is completed, the patient and family are instructed on the diagnosis and therapeutic options.
  • Once the treatment plan is developed, chemotherapy is administered, usually in the inpatient setting.
  • Following completion of the treatment cycle, patients are discharged home with detailed instructions for home care and with outpatient follow-up.
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Inpatient & Outpatient Medications

The following medications may be used:

  • Infection prophylaxis: Chemotherapy agents cause myelosuppression and immunosuppression. All patients should receive prophylaxis against Pneumocystis jiroveci with trimethoprim/sulfamethoxazole (trimethoprim 2.5 mg/kg/dose twice daily), administered on 3 consecutive days per week. Prophylaxis is started before chemotherapy and 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 intensity of chemotherapy has increased. Treat with 5-10 mcg/kg/d subcutaneously to start 24-36 hours after the last dose of chemotherapy. G-CSF is continued until the absolute neutrophil count is 2,000-10,000. See the Absolute Neutrophil Count calculator. A mouse model study by Agarwal et al suggested that G-CSF promotes the growth of neuroblastoma cancer stem cells that may be responsible for cancer relapse. [26, 27]
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Transfer

Management by primary care provider is as follows:

  • With oncology team supervision, routine care can be carried out by the primary care provider for patient convenience.
  • Monitoring of blood counts or chemistries and administration of blood products are common.
  • Some primary care providers with experience in the treatment of febrile neutropenia may be able to manage this complication of chemotherapy. Patients may quickly destabilize upon initiation of antibiotic therapy; thus, access to critical care services is required.
  • Maintain close contact with subspecialists and transfer the patient to the pediatric oncology center for any complications that may require specialized care.
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Deterrence/Prevention

The cause of neuroblastoma is unknown.

  • No specific environmental exposure or risk factors have been identified.
  • Currently, no specific recommendations on how to prevent this disease are known.
  • Screening for neuroblastoma in an attempt to diagnose high-risk patients earlier in the course of their disease has uncovered many patients with low-risk disease but has not had an impact on outcome in high-risk disease.
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Complications

The following complications may occur:

  • The most worrisome complication at disease presentation is cord compression from a paraspinal tumor. Evaluation of the patient by a neurosurgeon and consultation with a radiation oncologist are important.
  • In some individuals with neuroblastomas, early institution of chemotherapy is accepted if the tumor can be biopsied within 72 hours to make a diagnosis and to obtain necessary biologic studies. In the acute setting, chemotherapy may be as efficient as radiotherapy or laminectomy, and it may cause less morbidity. Treatment of cord compression with chemotherapy and steroids usually results in less complications; however, radiation therapy or surgery is often used as front-line treatment to prevent impending or progressive neurologic damage. In children who present with significant neurologic symptoms, none of these interventions assure a return of normal neurologic (motor) function.
  • Tumor lysis syndrome is unusual in neuroblastoma
  • Patients may present with severe hypertension or renal insufficiency, making initiation of chemotherapy, especially with platinum drugs, more difficult.
  • Myelosuppression and immunosuppression place the patient at risk of bleeding and infection. Febrile neutropenia is a medical emergency and requires immediate admission to the hospital and initiation of broad-spectrum antibiotic treatment.
  • After several cycles of therapy, depending on drugs administered, patients may develop impaired renal function, hearing loss, or delayed count recovery.
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Prognosis

Determinants of response and outcome include the following:

  • Stage, age, and several biologic characteristics of the tumor determine outcome.
  • Similarly, the patient may also have genetic polymorphism characteristics that influence drug absorption, distribution, metabolism, and excretion.

Several treatment strategies are available to treat patients with recurrent neuroblastoma.

  • A local recurrence in a patient with low-stage disease generally has a good prognosis, and patients usually receive standard chemotherapy, surgery, and/or radiation as necessary.
  • Patients with disseminated disease at presentation have a high recurrence rate and a poor outcome.
  • For patients with recurrent disease in this setting, various phase I/II agents are generally available.

Response criteria are used to evaluate the efficacy of therapy.

  • Complete clinical response - More than 90% decrease (sum of the products of the greatest perpendicular diameters) of the primary tumor and metastatic disease (if any), no new lesions, healing of bone lesions
  • Partial clinical response - A decrease of 50% or less (sum of the products of the greatest perpendicular diameters) of the primary tumor and metastatic disease (if any), no new lesions, healing of bone lesions
  • Minor response - More than 25% and less than 50% decrease (sum of the products of the greatest perpendicular diameters) of primary tumor and metastatic disease (if any), no new lesions, healing of bone lesions
  • No response - Less than 25% decrease (sum of the products of the greatest perpendicular diameters) of primary tumor or metastatic disease (if any), no new lesions
  • Progressive disease - More than 25% increase (sum of the products of the greatest perpendicular diameters) of the primary tumor or all metastatic lesions (if any), appearance of new lesions
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Patient Education

For compliance and good medical care, patients and families must understand the importance of treatment and adverse effects of medications used. In addition, they should learn to recognize and identify signs and symptoms of complications that require urgent medical care.

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