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Current Management of Childhood Ependymoma

Current Management of Childhood Ependymoma

Ependymoma is a rare central nervous system (CNS) tumor
in children, and our progress in treating this disease has been hampered by its
rarity as well as by a nonuniform approach to treatment among practitioners. Dr.
Merchant’s comprehensive review provides a framework for plotting a course of
further progress in treating children with ependymoma.

Optimal Local Control

As the author points out, optimal local control has been repeatedly shown to
be the single most important prognostic factor in the treatment of this disease,
and achieving this goal begins with maximal surgical resection. Failure to
perform a gross total or near total resection substantially decreases the
likelihood of long-term disease control.

To that end, one goal of the next Children’s Oncology Group trial is
emphasis on increasing the number of patients whose tumors are gross totally or
near totally resected. Second surgery is recommended for patients who undergo an
initial subtotal resection. In an attempt to facilitate the second surgery,
chemotherapy is administered between the first and second resections in the
hopes of rendering the tumor more amenable to complete removal and, perhaps,
reducing the likelihood of disease dissemination in the interim.

Following surgery, postoperative conformal radiation therapy is administered
to all patients, with the exception of those with supratentorial, differentiated
ependymomas whose tumors have been completely resected. Notably, and in contrast
to other contemporary trials, children will receive irradiation if they are 1
year of age or older at the time of planned irradiation. Merchant argues, based
on his own institutional experience, that in light of newer radiation
techniques, irradiation in young children will likely result in fewer long-term
neurologic sequelae than have been seen in the past, while improving long-term
survival. To date, disease control in his series has been excellent, but the
follow-up period is still relatively short.

Long-Term Neurologic Outcome

Aggressive attempts at local control appear to be a reasonable strategy to
improve the treatment of patients with ependymoma. However, we must be careful
to follow-up on the possible consequences of this approach. Although there is
reason to hope that the use of more modern radiation technology and
neurosurgical techniques will improve the long-term neurologic outcome of
survivors, this is by no means assured.

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