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The optic chiasm dose should be managed in a very

similar fashion to the spinal cord and should be defined on

CT or MR and appearing on at least two successive images.

If the cumulative treatment dose may exceed 54 Gy, the

chiasm should be excluded from the treatment after 54 Gy

and receive no more than 1.25 Gy per fraction at any point.

These guidelines also allow for the coverage of the target

volumes to be compromised after 54 Gy in selected cases.

Each cochlea should be contoured separately on the CT

data as a circular structure within the petrous portion of the

temporal bone. The size and position of the contoured

cochlea should be confirmed by viewing the structures in

three dimensions using the treatment planning system and

on two successive CT images. The mean dose to the

cochleae should be limited to 35 Gy. At these levels, the

risk of hearing loss is less than 5%

[ 25

].

The brainstem is central to the irradiated volume in

patients with posterior fossa tumors, and while major side

effects from radiation therapy have not been widely

reported, investigators remain very concerned about the

long-term effects of irradiation especially for children who

suffer neurological effects from tumor and surgery. Recent

data suggest that factors impeding neurologic recovery in

children with ependymoma treated with high-dose postop-

erative radiation therapy do not include radiation dose,

rather, the volume of tumor and clinical and treatment

factors related to tumor and surgery. Given the safety

profile of radiation therapy as administered in recent trials

and plans to further reduce the target volume for radiation

therapy, the risk of side effects involving the brainstem

should be further diminished

[ 26

].

Temporal lobe and whole brain doses of radiation

therapy are correlated with cognitive outcome corrected

for the age of the patient at the time of irradiation. This

important knowledge has driven investigators to find new

ways to reduce dose to normal tissues (shrinking target

volume margins) or this high-dose volume of irradiation

using conformal methods. Evaluating patients with ependy-

moma and considering radiation effects should not be

absent the potential effects of hydrocephalus

[ 18

,

27

].

Hypothalamic dose volume effects have been modeled

for patients with ependymoma suggesting that the risk of

endocrinopathy is low for most patients and that while even

low doses to the hypothalamus result in a risk for growth

hormone deficiency, other endocrinopathies are even less

common if baseline assessments prove to be normal.

Preexisting endocrine deficiencies in these children corre-

late with ventricle size (hydrocephalus) at diagnosis

[ 28

,

29

].

Result

The peer-reviewed scientific literature contains numerous

references to highly focused focal irradiation for intracra-

nial ependymoma in children; however, there is only one

perspective conformal series. The primary measure of

success for conformal radiation therapy is local tumor

control corrected for extent of resection which is the most

important prognostic factor. There are several contemporary

series that utilized fully or to a large extent conformal

radiation therapy. Local tumor control has been estimated at

68

89% when measured at 3

5 years (Table

1

)

[ 18

,

30 32

].

The rates of local control in the modern series are

considerably higher than those inferred from historic series

where event-free survival and not local control rates have

been reported (Table

2

)

[ 2 10 ]

.

IMRT

The use of IMRT in very young children has raised concern

about extraneous dose to normal tissues. Mansur et al.

[ 33

]

found that IMRT lowered peripheral doses near the target.

This was attributed to reduced internal scatter due to

smaller effective field sizes. The thyroid was given as an

example of a critical peripheral organ near to the targeted

volume. The peripheral dose was similar for both IMRT and

three-dimensional CRT indicating that peripheral dose was

difficult to predict by monitor units which are often

significantly greater for IMRT.

Table 1

Local tumor control estimates from contemporary reports using postoperative irradiation

Series

Time period

Patients

GTR (%)

Local control

MacDonald-PBRT

2000

2006

17

76

86% at 2 years

Schroeder-IMRT

1994

2005

22

77

68% at 3 years

Massimino-HFRT

1993

2001

46

74

70% at 4 years

Merchant-CRT/IMRT

1997

2007

153

85

89% at 5 years

PBRT

proton beam radiation therapy,

IMRT

intensity-modulated radiation therapy,

HFRT

hyperfractionated radiation therapy,

CRT

conformal

radiation therapy,

GTR

gross-total resection

Childs Nerv Syst