Chapter 54 Neuroblastoma
PATHOPHYSIOLOGY
Neuroblastomas are soft, solid tumors originating from neural crest cells that are precursors of the adrenal medulla and sympathetic nervous system. Neuroblastomas can occur wherever sympathetic nervous tissue is found. The majority of tumors are usually in the abdomen, either in the adrenal gland or the sympathetic ganglia. Less common primary sites include the paraspinal area of the thorax, the neck, and the pelvis. Neuroblastomas often impinge on adjacent tissues and organs and can metastasize to the lymph nodes, bone, bone marrow, and/or subcutaneous tissue. Researchers are studying genetic mutations in the neuroblastoma cells as they seek to identify the etiology of neuroblastoma.
INCIDENCE
1. Neuroblastoma is the most common extracranial solid tumor of childhood and the most common neoplasm of infants.
2. It is the second most common type of childhood tumor.
3. Approximately 650 new cases are diagnosed each year in the United States.
4. The estimated incidence is 1 in 7000 births.
5. Neuroblastoma most commonly occurs in children between birth through the fourth year of life.
6. The unique phenomenon of spontaneous tumor regression and maturation into benign forms may allow many cases of neuroblastoma to go undetected.
7. Survival rates are 90% in low-risk patients, 70% to 90% in intermediate-risk patients, and 30% in high-risk patients (see Medical and Surgical Management section in this chapter).
CLINICAL MANIFESTATIONS
COMPLICATIONS
1. At diagnosis: Depending on the location of the tumor, neuromuscular complications can include lower-extremity weakness or paralysis. A hematologic complication is metastasis to lymph nodes, bone, and bone marrow.
2. During treatment: Adverse events from chemotherapy, radiation therapy, and/or surgery can be life-threatening. These can include infection and organ toxicities.
LABORATORY AND DIAGNOSTIC TESTS
1. Complete blood count—to detect anemia caused by many secondary factors (e.g., hemorrhage, disseminated intravascular coagulation)
2. Urinary levels of catecholamines (vanillylmandelic acid and homovanillic acid)—tumor markers that are elevated owing to overproduction by tumor cells or defective storage within tumor cells
3. Ferritin level—increase correlates with poorer prognosis
4. Neuron-specific enolase level—elevated owing to correlation with amount of active neuronal tissue
5. Bilateral bone marrow aspiration and biopsies—to reveal marrow involvement, confirm diagnosis, and allow staging
6. Chest radiographic study—to delineate primary thoracic neuroblastoma and vertebral and paravertebral involvement
7. Computed tomography and magnetic resonance imaging—to determine extent of disease
8. Bone scan—to assess for metastasis
9. Metaiodobenzylguanidine (MIBG) scan—to assess involvement of bone and tissue. An MIBG scan provides a scintigraphic image of neuroendocrine tumors.
10. Tumor biopsy—for pathologist to examine tumor tissue and provide diagnosis. Biology studies provide information on which risk category is based. These studies include the following elements:
MEDICAL AND SURGICAL MANAGEMENT
The international staging system for neuroblastoma (INSS) standardizes definitions and categorizes disease according to radiographic and surgical findings, plus bone marrow status. Localized tumors are divided into stages 1, 2A, and 2B depending on the extent of tumor excision and the status of regional lymph nodes. In stage 3, the tumor crosses the vertical midline of the body (marked by the spine) and cannot be removed surgically, or the tumor is restricted to one side of the body but there are lymph nodes on the opposite side of the body that are positive for cancer. The patient with stage 4 neuroblastoma has had the disease spread to distant lymph nodes, bone, bone marrow, liver, skin, and/or other organs. Stage 4S is a unique stage; the “S” stands for “special.” These tumors often spontaneously regress without any treatment. The child with stage 4S must be less than 12 months of age and have a localized primary tumor that has spread only to the skin, the liver, or the bone marrow.
The Children’s Oncology Group, the pediatric cancer cooperative study group in North America, is investigating a system for assigning patients to treatment according to one of three risk groups, low, intermediate, or high. The risk is based on the child’s INSS stage, age, MYCN status, Shimada histology, and DNA ploidy.
Children with a low-risk tumor are generally treated with surgical resection. Stage 2 low-risk tumors are treated with chemotherapy only if less than 50% of the tumor has been removed. In the other low-risk children, chemotherapy is recommended only for life-threatening symptoms that cannot be relieved by safe surgical resection of the tumor. Chemotherapy agents include carboplatin, cyclophosphamide, doxorubicin, and etoposide, given in moderate doses. The treatment of children with low-risk 4S neuroblastoma is determined by the child’s symptoms.
Intermediate-risk tumors are treated with moderate doses of carboplatin, cyclophosphamide, doxorubicin, and etoposide given for 12 to 24 weeks. Surgical resection may be done before starting chemotherapy, or, if surgery cannot be performed safely, it may be delayed until the completion of chemotherapy.
Children who are in the high-risk group are treated aggressively with high doses of combination chemotherapy. Agents used include cyclophosphamide, ifosfamide, cisplatin, carboplatin, vincristine, doxorubicin, and etoposide. After the primary tumor has been shrunk with induction chemotherapy, surgical resection is performed. Children are then treated with myeloablative chemotherapy and stem cell rescue (i.e., bone marrow and/or peripheral blood stem cell transplantation). Radiation treatment is given to the primary tumor site. Some children may also receive radiation to sites of metastasis. After recovery, children are treated with the biologic modifier oral 13-cis-retinoic acid for 6 months. Current research is investigating the use of purged peripheral blood stem cells for use in the stem cell rescue procedure. Immunologic interventions that are currently being studied use monoclonal antibodies to the GD2 ganglioside, an antigen expressed on the surface of neuroblastoma cells.
NURSING ASSESSMENT
1. Refer to Appendix A for system-specific assessments.
2. Be aware that physical assessment depends on tumor site and related system. Palpation of tumor site should be avoided.
3. Be aware that assessment of the child with neuroblastoma should encompass all aspects of medical treatment, including chemotherapy, surgery, radiation, and stem cell transplantation.
4. Assess child for verbal and nonverbal expressions of pain (see Appendix I).
NURSING DIAGNOSES
NURSING INTERVENTIONS
Surgical Phase
1. Prepare child for clinical staging procedures with age-appropriate approach (see the Preparation for Procedures or the Surgery section in Appendix F).
2. Monitor for signs of infection including fever, chills, lethargy, erythema, and/or drainage at central line site and/or surgical site.
3. Monitor respiratory function including respiratory rate and effort, lung auscultation, and oxygen saturation.
4. Provide fluid and electrolyte management including the following: monitor intake and output (I&O), monitor electrolytes, replace drainage and electrolytes per order, assess chemistries, assess skin turgor and mucous membranes, and assess for edema.
Chemotherapy and/or Radiation Phase
1. Assess tumor site using observation and inspection; palpation is contraindicated.
2. Minimize side effects of multiagent chemotherapy and radiation therapy. Refer to Table 49-1 in chapter on leukemia: Nursing Interventions Related to the Child Undergoing Chemotherapy and Radiotherapy.
3. Observe for medication or transfusion reactions.
4. Assess integrity of skin and mucous membranes.
5. Monitor for signs of infection including fever, chills, lethargy, erythema, and/or drainage at central line site.
6. Monitor physical and emotional growth and development of child (see Appendix F).
7. Teach parents about medications their child is receiving (see Appendixes B and E).
8. Refer child and/or siblings to child life specialist.
9. Assess for pain using age-appropriate technique (see Appendix I).
Discharge Planning and Home Care
Instruct parents about home care management:
1. Signs of infection and guidelines on when to seek medical attention.
2. Postoperative wound care after tumor excision including assessment of wound site, cleansing site, and changing dressing.
3. Care of child’s central venous access device, including site care, dressing change, flushing, cap change, and emergency care.
4. Adherence with treatment and medical appointments.
5. Facilitate understanding of the action of the medications and monitoring of their untoward effects.
6. Ensure the child’s nutritional needs are met including use of home enteral nutrition.
7. Observe for potential behavioral changes in child and/or siblings following hospitalization and during long-term management of the child’s medical needs. Direct families in activities to support ongoing growth and development.
8. Refer family to social services for support and resource utilization (see Appendix G).
CLIENT OUTCOMES
1. Child and family will demonstrate ability to cope with life-threatening illness.
2. Child will be free of infection.
3. Child and family will understand home care and long-term follow-up needs.
4. Child will progress in achieving developmental milestones.
5. Child will maintain/exceed body mass index (BMI) baseline status.
6. Child will be free of pain.
7. Child will resume prehospital and/or pretreatment levels of activity.
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Curesearch [Neuroblastoma (website)] www.curesearch.org/for%5Fparents%5Fand%5Ffamilies/newlydiagnosed/article.aspx?ArticleId=145&StageID=1&TopicId=1&Level,=1. Accessed March 9, 2006
Dadd G. Neuroblastoma. Baggott CR, et al. Nursing care of children and adolescents with cancer, ed 3, Philadelphia: WB Saunders, 2002.
Duffey-Lind E. Neuroblastoma. Kline NE, ed. Essentials of pediatric oncology nursing: A core curriculum, ed 2, Glenview, IL: Association of Pediatric Oncology Nurses, 2004.
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National Cancer Institute. Neuroblastoma (PDQ) Health Professional Version. (website) www.cancer.gov/cancertopics/pdq/treatment/neuroblastoma/HealthProfessional/page1 Accessed March 9, 2006
