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Pediatric Medulloblastoma Follow-up

  • Author: Tobey J MacDonald, MD; Chief Editor: Max J Coppes, MD, PhD, MBA  more...
 
Updated: Nov 26, 2014
 

Further Outpatient Care

Radiotherapy

Daily outpatient radiotherapy (usual dose fractions of 180 cGy/d) is performed for approximately 6 weeks.

Physical and neurologic examination

Careful monitoring of response and treatment-associated side effects is performed weekly during radiotherapy and at least every 2 weeks during chemotherapy.

Reevaluation immediately before each cycle of chemotherapy is necessary to document resolution of previous treatment-related toxicities.

Following the completion of therapy, assessments are conducted every 3 months for the first 12-18 months, every 6 months for the next 2 years, and then annually, provided no complications have occurred.

Imaging studies

To have an objective measurement of tumor response to therapy, MRI with contrast of the head is performed at the completion of radiotherapy, after every 2 cycles of chemotherapy, and at the end of therapy.

Unless clinically indicated, follow-up MRI scans after the completion of therapy are performed in conjuncture with the physical and neurologic examination schedule.

MRI with contrast of the spine is performed only at the completion of therapy and annually thereafter unless metastatic spinal disease was observed, in which case more frequent evaluation may be necessary.

Laboratory studies

Carefully monitor toxicities associated with therapy. Weekly CBC counts are necessary during radiotherapy and chemotherapy, as well as liver function studies, electrolytes, renal function, and a hearing test before each cycle of chemotherapy and again at the end of treatment.

Baseline studies should be performed before the initiation of any therapy. These tests may need to be performed annually for the first 3-5 years after therapy.

A baseline endocrinologic and neuropsychologic evaluation should be performed at the completion of therapy and annually thereafter.

Additional tests for the purposes of monitoring specific investigational protocol treatment-related toxicity (eg, echocardiogram, pulmonary function tests, other studies) may be required according to protocol guidelines.

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Further Inpatient Care

Admit patients with medulloblastoma for specific chemotherapy and for complications (eg, fever, neutropenia, infection) as a result of therapy.

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Inpatient & Outpatient Medications

Inpatient medications are dictated by the most current chemotherapeutic protocols available for the treatment of medulloblastoma. The most commonly used chemotherapeutic agents are DNA alkylators.

Most regimens require the concomitant use of an antiemetic.

Because of the immunosuppressive effects of chemotherapy, trimethoprim sulfamethoxazole and nystatin are commonly prescribed for prophylaxis against Pneumocystis carinii pneumonia and mucocutaneous candidiasis, respectively, for the duration of treatment.

Granulocyte colony stimulating factor (GCSF) following chemotherapy may be used in treatment regimens expected to cause marked neutropenia.

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Transfer

Transfer to centers that can provide appropriate MRI imaging studies, neurosurgical intervention, radiotherapy, and chemotherapy may be necessary.

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Deterrence/Prevention

Avoid exposure to ill contacts during therapy.

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Complications

Complications include the following:

  • Obstructive hydrocephaly
  • Neurologic impairment
    • Holland et al conducted a study to identify factors associated with academic difficulties in survivors of pediatric medulloblastoma.[10] The study focused on school competence and fluent academic performance. Thirty-six patients (aged 7-18 years) were recruited for the study. Participants completed a neuropsychological screening battery that included selected Woodcock-Johnson III Tests of Achievement subtests. Parents completed the Child Behavior Checklist. Basic academic skill development was found to be broadly average, in contrast to fluent academic performance, which was significantly worse than average. The investigators concluded that school competence may have utility in estimating levels of educational success in survivors of pediatric medulloblastoma and that academic difficulties in these patients may be better assessed through measures of deficits in fluent academic performance rather than academic skills.[10]
  • Pain secondary to metastasis
  • Chemotherapy-induced effects
    • Anemia
    • Thrombocytopenia and increased risk for bleeding
    • Neutropenia and increased risk for life-threatening bacterial, viral, and fungal opportunistic infections
    • Nephrotoxicity, ototoxicity, hepatotoxicity, and neurotoxicity
  • Radiation-induced effects
    • Neurocognitive and endocrinologic dysfunction
    • Mineralizing microangiopathy with ischemia or infarct
    • Secondary CNS and thyroid malignancies
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Prognosis

The clinical criteria used to assign prognostic risk groups is continuing to evolve but is currently based on the following 3 features: age, metastasis at presentation, and extent of postoperative residual disease.

Metastasis stage (M stage) is derived from the Chang classification staging system.[11] The M stage classification is as follows:

  • M0 - No gross subarachnoid or hematogenous metastasis
  • M1 - Microscopic tumor cells found in CSF
  • M2 - Gross nodular seeding in cerebellum, cerebral subarachnoid space, or in the third or fourth ventricles
  • M3 - Gross nodular seeding in spinal subarachnoid space
  • M4 - Extraneuraxial metastasis.

The CCG-921 study confirmed prospectively the unequivocal impact of metastasis at diagnosis on early tumor progression or relapse.[12] Although the M1 stage lacked statistical power in this study, the lower M stage correlated with improved progression-free survival rates (M0 > M1 > M2+) in children older than 3 years.

The current risk groups are specifically defined, as follows:

  • Average-risk disease is defined as patients older than 3 years who are at stage M0 with less than 1.5 cm 2 of residual tumor postoperatively. The 5-year survival rate for this group is currently 78%.
  • Poor-risk disease is defined as patients older than 3 years who are at stage M1-M4 and/or with more than 1.5 cm 2 of residual tumor postoperatively. The 5-year survival rate for this group is currently 30-55%. Those with anaplastic tumors are also considered poor-risk.
  • All patients with nonposterior fossa tumors that are morphologically similar to medulloblastoma (primitive neural ectodermal tumors [PNETs]) have a poor prognosis similar to patients with poor-risk medulloblastoma, regardless of dissemination.
  • Infants are defined as patients younger than 3 years. This group has the worst prognosis, regardless of M stage and extent of postoperative residual disease. The 5-year survival rate is approximately 30%; however, patients with metastatic disease do considerably worse.

Biologic tumor markers will likely supplement stratification in the future.

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Patient Education

Patients and family members should be instructed about the care of the central venous catheter.

Patients should be instructed about protection against infection and what to do should infection be suspected during therapy.

For excellent patient education resources, visit eMedicineHealth's Cancer Center. Also, see eMedicineHealth's patient education article Brain Cancer.

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Contributor Information and Disclosures
Author

Tobey J MacDonald, MD Professor, Department of Pediatrics, Emory University School of Medicine; Director, Pediatric Brain Tumor Program, Aflac Chair for Neuro-Oncology, Aflac Cancer and Blood Disorders Center, Children's Healthcare of Atlanta

Tobey J MacDonald, MD is a member of the following medical societies: American Association for Cancer Research, Society for Neuro-Oncology, International Society of Paediatric Oncology

Disclosure: Nothing to disclose.

Coauthor(s)

Roger J Packer, MD Senior Vice President, Neuroscience and Behavioral Medicine, Director, Brain Tumor Institute, Children’s National Medical CenterProfessor of Neurology and Pediatrics, The George Washington University

Roger J Packer, MD is a member of the following medical societies: American Academy of Neurology, American Neurological Association, American Pediatric Society, Child Neurology Society, Children's Oncology Group, Society for Neuro-Oncology, Pediatric Brain Tumor Consortium, Neurofibromatosis Clinical Trials Consortium

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Steven K Bergstrom, MD Department of Pediatrics, Division of Hematology-Oncology, Kaiser Permanente Medical Center of Oakland

Steven K Bergstrom, MD is a member of the following medical societies: Alpha Omega Alpha, Children's Oncology Group, American Society of Clinical Oncology, International Society for Experimental Hematology, American Society of Hematology, American Society of Pediatric Hematology/Oncology

Disclosure: Nothing to disclose.

Chief Editor

Max J Coppes, MD, PhD, MBA Executive Vice President, Chief Medical and Academic Officer, Renown Heath

Max J Coppes, MD, PhD, MBA is a member of the following medical societies: American College of Healthcare Executives, American Society of Pediatric Hematology/Oncology, Society for Pediatric Research

Disclosure: Nothing to disclose.

Additional Contributors

Kathleen M Sakamoto, MD, PhD Shelagh Galligan Professor, Division of Hematology/Oncology, Department of Pediatrics, Stanford University School of Medicine

Kathleen M Sakamoto, MD, PhD is a member of the following medical societies: International Society for Experimental Hematology, American Society of Hematology, American Society of Pediatric Hematology/Oncology, Society for Pediatric Research

Disclosure: Nothing to disclose.

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MRI showing a medulloblastoma of the cerebellum.
Section displaying Homer-Wright rosettes and pseudorosettes of a medulloblastoma.
This section displays a typical medulloblastoma, composed of undifferentiated cells with deeply basophilic nuclei of variable size and shape and little discernible cytoplasm.
 
 
 
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