Pediatric Medulloblastoma Workup

  • Author: Tobey MacDonald, MD; Chief Editor: Max J Coppes, MD, PhD, MBA   more...
 
Updated: Mar 1, 2012
 

Laboratory Studies

The routine pretreatment laboratory evaluation for medulloblastoma includes CBC count, electrolytes, liver, and renal function tests.

Baseline thyroid function studies and viral titers are also recommended.

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Imaging Studies

CT scanning

A CT scan of the head with and without contrast has more than 95% sensitivity for the detection of brain tumors.

On CT scans, prominent hydrocephalus and a solid, homogeneous, isodense to hyperdense, contrast-enhancing, midline cerebellar mass are characteristic of (although not diagnostic of) medulloblastoma.

MRI

Head and spinal MRI with and without gadolinium should be performed in all patients with CT or clinical findings consistent with medulloblastoma.

Other midline posterior fossa tumors, such as cerebellar astrocytoma and ependymoma, may have a similar appearance on CT.

MRI can be useful in such instances by better demonstrating the anatomic origin and extent of tumor (see the image below).

MRI showing a medulloblastoma of the cerebellum. MRI showing a medulloblastoma of the cerebellum.

Preoperative and postoperative MRI is required for detection and measurement of residual disease following surgical resection. Postoperative MRI evaluation should be performed within 72 hours of surgery to delineate residual tumor from the postsurgical inflammatory changes that are visualized on MRI at this time.

Spinal MRI is the most sensitive method available for detection of spinal cord metastasis.

Bone scan

Because medulloblastoma can metastasize outside the CNS, especially to bone, a bone scan with plain film correlation may be useful in symptomatic patients.

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Other Tests

A baseline hearing test (audiography or brainstem auditory-evoked response [BAER]) is recommended because of the potential toxicity from radiation and chemotherapy.

Some investigational treatment protocols may require additional tests, such as echocardiography, pulmonary function tests, or other more specific tests, for the purposes of monitoring treatment-related toxicity.

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Procedures

Lumbar puncture

CSF cytologic examination is useful for the detection of microscopic leptomeningeal tumor dissemination. However, neither clinical symptoms nor negative CSF cytologic findings can be relied on to indicate the presence of nodular spinal cord disease. As many as 50% of patients with positive spine MRI studies are asymptomatic and have negative cytologic results.

Funduscopic examination (or CT or MRI) must be performed before lumbar puncture (LP) to rule out the presence of hydrocephaly.

In known cases of medulloblastoma, LP generally is deferred until 2 weeks postoperation to avoid the presence of tumor cells that have disseminated as a result of surgery.

Bone marrow aspirate and biopsy

Medulloblastoma rarely metastasizes to bone marrow.

These tests should be reserved for patients who demonstrate abnormal peripheral blood findings that have no clear etiology.

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Histologic Findings

Medulloblastomas are undifferentiated embryonal neuroepithelial tumors of the cerebellum. They are highly cellular, soft, and friable tumors composed of cells with deeply basophilic nuclei of variable size and shape, little discernible cytoplasm, and often abundant mitoses (see the image below).

This section displays a typical medulloblastoma, cThis section displays a typical medulloblastoma, composed of undifferentiated cells with deeply basophilic nuclei of variable size and shape and little discernible cytoplasm.

These characteristics give the microscopic appearance of a small, round, blue cell tumor. Morphologically identical tumors arising in the pineal region are termed pineoblastomas, and those arising in other CNS locations are called primitive neuroectodermal tumors (PNETs).

Homer-Wright rosettes (ringlike accumulations of tumor cell nuclei around a neuropil-containing or fibrillary core) and pseudorosettes are variably present (see the image below).

Section displaying Homer-Wright rosettes and pseudSection displaying Homer-Wright rosettes and pseudorosettes of a medulloblastoma.

These tumors express neuronal and neuroendocrine markers, including synaptophysin and neurofilament proteins.

Various degrees of glial or neuroblastic differentiation are noted, suggesting that the primitive cell of origin possesses the capacity for bipotential differentiation. A histologic variant with abundant stromal component, desmoplastic medulloblastoma, occurs dominantly in the lateral cerebellar areas of adolescents and adults. Another more recently described variant is characterized by marked features of anaplasia that is associated with MYCC oncogene amplification.

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Contributor Information and Disclosures
Author

Tobey MacDonald, MD  Clinical Director of Neuro-Oncology, Children's Hospital National Medical Center; Associate Professor, Department of Pediatric Hematology-Oncology, George Washington University

Tobey MacDonald, MD is a member of the following medical societies: American Association for Cancer Research, Children's Oncology Group, Pediatric Brain Tumor Consortium, and Society for Neuro-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, Neurofibromatosis Clinical Trials Consortium, Pediatric Brain Tumor Consortium, and Society for Neuro-Oncology

Disclosure: Nothing to disclose.

Specialty Editor Board

Kathleen M Sakamoto, MD, PhD  Professor and Chief, Division of Hematology-Oncology, Vice-Chair of Research, Mattel Children's Hospital at UCLA; Co-Associate Program Director of the Signal Transduction Program Area, Jonsson Comprehensive Cancer Center, California Nanosystems Institute and Molecular Biology Institute, University of California, Los Angeles, David Geffen School of Medicine

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

Disclosure: Nothing to disclose.

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, American Society of Clinical Oncology, American Society of Hematology, American Society of Pediatric Hematology/Oncology, Children's Oncology Group, and International Society for Experimental Hematology

Disclosure: Nothing to disclose.

Samuel Gross, MD  Professor Emeritus, Department of Pediatrics, University of Florida; Clinical Professor, Department of Pediatrics, University of North Carolina; Adjunct Professor, Department of Pediatrics, Duke University

Samuel Gross, MD is a member of the following medical societies: American Association for Cancer Research, American Society for Blood and Marrow Transplantation, American Society of Clinical Oncology, American Society of Hematology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Chief Editor

Max J Coppes, MD, PhD, MBA  Senior Vice President, Center for Cancer and Blood Disorders, Children's National Medical Center; Professor of Medicine, Oncology, and Pediatrics, Georgetown University School of Medicine; Clinical Professor of Pediatrics, George Washington University School of Medicine and Health Sciences

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