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MEDULLOBLASTOMA

Medulloblastomas are tumors that arise from primitive neural cells and are almost exclusively found within the Cerebellum (the balance and coordination center of the Brain located in the Posterior Cranial Fossa) in children. They account for up to 10 percent of primary Brain tumors and are one of the most common tumors in the Posterior Fossa. Medulloblastomas account for almost 20% of Central Nervous System tumors in patients under the age of 20.


Figure 1A (Left): MRI Scan (Sagittal View-Gadolinium Enhanced) of a Young child with a large 4th Ventricular Medulloblastoma (Vertical Arrow) with compression of the Cerebellum (Curved Arrow).

Figure 1B (Center): MRI Scan (Coronal View-same patient). The tumor has considerable "enhancement" indicating a substantial blood supply.

Figure 1C (Right): MRI Scan (Transaxial View-same patient). The tumor (Arrow) fills the 4th Ventricle demonstrates extensive "enhancement".

There are similar tumors occurring in adults, although these are quite rare. Neuropathologists tend to regard these as "Blue Cell Tumors" (based upon their staining characteristics when specimens are prepared for viewing under a microscope) or "Cerebellar Sarcomas". We have five (5) such patients (See Figures 2 A & B) in our series with long term follow of from 5 years (in 2 patients) and over 15 years in two (2) other individuals.


Figure 2A (Left): MRI Scan (Coronal View) A large Medulloblastoma in a 35 year old Male. Although the tumor has a Cyst within it (Arrow), it proved to be quite a vascular lesion.

Figure 2B (Right): MRI Scan (Transaxial View-same patient) Note the "white" area that identifies Cerebellar swelling (edema) adjacent to the tumor indicated by the Arrow.


SYMPTOMS

Most Medulloblastomas arise in and occupy the Fourth Ventricle. The result is the development of increased intracranial pressure consequent to the ensuing Hydrocephalus. Morning (or matutinal) headaches are a common initial symptom (since intracranial pressure usually rises to some degree in everyone when lying flat and sleeping.) These headaches often disappear once the patient is up and about for a short while. Symptoms such as listlessness, lethargy, vomiting in the early morning when headaches are at a maximum level, balance and gait problems, dizziness and double vision, are quite common for these tumors.

DIAGNOSIS

MRI scans are the most helpful neuroimaging study in identifying a Posterior Cranial Fossa tumor. (See Figures 1 & 2.)

TREATMENT

Surgery

Direct surgical resection and "debulking" of the tumor is mandatory as the initial step in the management of patients with Medulloblastomas. As much tumor as possible is removed, without causing additional neurological injury.

Radiation Therapy

Radiation Therapy is the single most important adjunct, once the diagnosis of Medulloblastoma has been established by surgical resection. Radiation Therapy is avoided for children less than two (2) years of age since the young, developing Brain tolerates large doses of radiation poorly.

Consideration must be given for this tumor's potential to "seed" throughout the Cerebrospinal Fluid (CSF) pathway such as within the Spinal Canal. This may warrant Spinal Axis radiation on a prophylactic basis.

Chemotherapy

Chemotherapy is a primary adjunctive treatment for patients two (2) years old and under since radiation is contraindicated in that age group. Chemotherapy is the third arm of this comprehensive effort to prolong and maintain a high quality of life. Radiation therapy and Chemotherapy (with agents such as CCNU, vincristine and cis-platinum) following soon after aggressive surgical resection appears to offer significant improvement in survival rates for this very deadly disease. These medications are generally considered to be "traditional management" ("standard" forms of chemotherapy.) Currently there are some unconventional chemotherapeutic alternatives that offer considerable hope for improved quality and length of survival. One of our Neuro-oncologists has utilized these newer medications such as Temodar (temozolamide), Avastin (bevacizumab, an anti-angiogenesis agent) and CPT-11, either alone (or more commonly) in combination or with other drugs, to produce encouraging results. Additional information regarding these treatments is available at the "Virtual Trials" website.


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This page last edited on 2/19

All content ©2016 by Neurosurgical Consultants, P.A.
Author, Martin L. Lazar, MD, FACS
All Rights Reserved. See Usage Notices.