. they account for 2% to 5% of all primary intracranial neoplasms and 5.6% to 13% of intracranial tumors in children17 ) .
they are classified as intracranial tumors of benign or unspecified behavior by pediatric cancer registries8 ) .
current treatment strategies for craniopharyngiomas include cystic drainage , intratumoral chemotherapy , limited resection , or a combination of gross total resection ( gtr ) and radiation therapy ( rt ) .
surgery remains the treatment of choice because it allows rapid decompression , minimizes recurrence and provides a histological diagnosis .
however , surgery can produce high treatment - related morbidity such as panhypopituitarism , diabetes insipidus , hypothalamic obesity , cognitive deficits4,10 ) , or even death due to the close proximity of crucial neurovascular structures .
furthermore , secondary surgery to treat recurrent craniopharyngioma is associated with a higher risk of complications and a lower cure rate6 ) .
subtotal resection ( str ) combined with rt and radiosurgery ( gkrs ) is being used increasingly as either a primary or secondary treatment for patients with craniopharyngioma2,12 ) .
the clinical records of pediatric patients treated for craniopharyngiomas between december 1995 and february 2011 were reviewed .
thirty - five pediatric patients diagnosed with a craniopharyngioma were treated , and their medical records and imaging data were analyzed retrospectively .
inclusion criteria were as follows : 1 ) age less than 19 years , and 2 ) surgical resection with a histopathological diagnosis of craniopharyngioma .
the initial tumor diameter was calculated as the average of the longest diameter and the two other diameters vertical to the longest one .
the extent of resection was determined by analyzing postoperative computed tomography scans and magnetic resonance imaging scans .
a clinical and radiologic follow - up was performed three months after the diagnosis or initial treatment and then at intervals of one to two years thereafter .
progression of craniopharyngioma was defined as tumor growth on sequential imaging with or without associated symptoms .
the overall survival and progression - free survival rates were estimated using the kaplan - meier method .
statistical analyses were performed using the statistical package for the social sciences ( spss , version 18.0 , spss inc . , chicago , il , usa ) .
the mean follow - up duration was 76 months ( range , 10 - 195 ) .
the female - to - male ratio was 16 : 19 , and the mean age was 8.6 years ( range , 1 - 17 ) .
the initial mean tumor diameter was 30.4 mm ( range , 14 - 55 mm ) .
preoperative tumors were divided into three types : cystic ( 15 patients ) , solid ( 13 patients ) and mixed ( 7 patients ) . the preoperative tumor location was classified according to sami 's classification16 ) .
eight patients were classified as grade 1 ( intrasellar or infradiaphragmatic ) and grade 2 ( occupying the cistern ) ; 8 patients were classified as grade 3 ( lower half of the third ventricle ) ; 13 patients met grade 4 criteria ( upper half of the third ventricle ) ; and 6 patients were grade 5 ( reaching the septum pellucidum or lateral ventricle ) .
the symptoms at initial presentation included visual symptoms ( 13 patients ) , such as a visual field defect and decreasing visual acuity , nausea / vomiting ( 8 patients ) , endocrine abnormalities ( 7 patients ) , headaches ( 3 patients ; 42.9% ) , dizziness ( 3 patients ) , and seizure ( 1 patient ) .
baseline characteristics of the 35 patients stratified by initial treatment are summarized in table 1 .
initially , gtr was performed in 30 patients and str followed by rt was administered in 5 patients .
the rt dosage of patients who underwent str was 5400 cgy with a median fraction size of 180 cgy .
twenty - eight patients had endocrine abnormalities that required endocrine hormonal replacement post - treatment .
the frequency of diabetes insipidus in patients undergoing gtr was 56.7% compared with 100% in patients who underwent str combined with postoperative rt .
the difference in endocrine requirements and complications among the groups was not statistically significant because of the small number of cases ( table 2 ) .
progression - free was 51.2 months ( range , 3 - 182 ) : 49.7 months in the patients who underwent gtr and 60.2 months in those who received str followed by rt ( table 3 ) .
overall survival and local control rates at 10 years were 94.75.1% and 37.111.9% , respectively ( fig .
the mean time from the initial diagnosis until recurrence after surgery was 32.4 months ( range , 5 - 108 ) .
differences in tumor type and calcification were not significant in patients who had a recurrence . of 14 cases with a recurrence after gtr , 5 patients were treated with second surgery ( gtr ) , 1 patient with rt , 4 patients with gkrs , and 4 with str followed by rt .
no recurrence had developed during the time from rt until the last follow - up .
the remaining four patients underwent str followed by rt ; their mean dosage was 3810 cgy ( range , 1040 - 5400 cgy ) .
leksell gamma knife types b or c ( elekta instruments , atlanta , ga , usa ) were used for radiosurgery .
the mean marginal dosage was 11.2 gy ( range , 9 - 14 gy ) , and the mean target volume was 1719 mm ( range , 424 - 6874 ) .
the local control rate of the lesions treated with gkrs was 100% ( decreased in two patients and stable in two patients ) at the final follow - up ( mean , 75 months ) .
the local tumor control rates for patients who received rt or gkrs was 100% at relapse ( p=0.00 ) ( fig .
two cases with recurrence after str followed by rt as an initial treatment were treated with gtr via a transnasal approach .
no recurrence had developed during the time from repeat gtr until the last follow - up .
current treatment strategies for craniopharyngiomas include cystic drainage , intratumral chemotherapy , limited resection or gtr , and rt .
radical resection at presentation offers the best chance of disease control and potential cure with acceptable morbidity3 ) because craniopharyngiomas are histologically benign .
however , surgery rarely obliterates the tumor completely . the ability to achieve gtr varies between 43 - 76% in published pediatric series4,10 ) .
furthermore , local recurrence rates have been reported to range from 20 - 30% after gtr6,9 ) and from 70 - 100% after str without adjuvant treatment10,20,21 ) . to prevent recurrence ,
aggressive resection is required , which may result in severe permanent neurologic injury and related complications , such as post - treatment endocrinopathy5,7 ) , hypothalamic and frontal lobe damage and hypothalamic obesity4,10 ) . in our series
the local recurrence rate ( 14 patients , 46.7% ) was slightly high compared with previous reports .
the cause of our high local recurrence rate may be a minimal capsule or calcification around the tumor , which was closely adjacent to critical structures .
the fine balance between decreasing neurological deficits and controlling local tumors has led to the use of radiation .
radiation therapies , including fractionated radiation and stereotactic radiosurgery , are often applied postoperatively in the event of str or tumor recurrence2,7,11,12,18,19 ) .
fractionated rt improves craniopharyngioma control and survival18 ) and is the standard treatment for residual or recurrent tumors .
most patient series have demonstrated that when combined with str , adjuvant radiotherapy allows for greater tumor control and survival than surgery alone19 ) .
dosages of 5000 - 6000 cgy are most commonly used19 ) . in our study , five patients underwent fractionated rt combined with str as an initial treatment .
the mean dosage was 5400 cgy with a median fraction size of 180 cgy , and the mean tumor size was 37.7 mm ( range , 25 - 45 mm ) .
the mean time to recurrence ( 16.0 months ; range , 3 - 29 ) was shorter compared with that of gtr .
of 14 cases with a recurrence after first surgery ( gtr ) , 9 patients were treated with second surgery .
however gtr could be achieved only in 5 patients due to adhesion to surrounding critical structures .
five patients who experienced recurrence of their tumor after gtr as an initial treatment underwent rt as a secondary treatment
no recurrence developed from the time of rt treatment through the last follow - up .
stereotactic radiosurgery is a relatively recent therapeutic option for craniopharyngioma that has significantly improved the effectiveness of and morbidity associated with rt .
kobayashi11 ) published the largest treatment and outcomes series , which involved 98 cases . at a mean marginal dosage of 11.5 gy and a mean tumor size of 3.5 cm ,
the tumor control rate was 79.6% with a complete response in 19.4% and a partial response in 67.4% of the cases .
the actual five- and ten - year survival rates were 94.1% and 91% , respectively , with respective pfs rates of 60.8% and 53.8% .
young age was reported to be a predictor of unfavorable outcome after radiosurgery for craniopharyngioma . in our study , four patients received gkrs .
the mean marginal dosage was 11.2 gy ( range , 9 - 14 gy ) , and the mean target volume was 1719 mm ( range , 424 - 6874 ) .
the local control rate of the lesions treated with gkrs was 100% ( decreased in two patients and stable in two patients ) at the final follow - up ( mean , 75 months ) , although all patients were young .
nevertheless , the use of rt in malignant lesions in children has always posed a therapeutic dilemma ; the risk of long - term toxicity must be balanced with the risk of recurrence .
this issue is particularly challenging in the management of a benign neoplasm , such as craniopharyngioma , for which overall survival rates are 90 - 95% at five years and for which side effects from therapy or local progression of the disease can have devastating consequences5 ) .
treatment - related side effects of rt have been well described in the literature and include endocrine , visual and cognitive sequelae as well as vasculopathy and secondary malignancies1,9,13 ) .
side effects may occur acutely , but they are generally considered to be insidious9 ) . in our study , no newly developed side effects or complications associated with rt or gkrs , such as visual disturbances , endocrine disorders or decreased cognitive function , were observed .
this may be because all patients underwent surgery as an initial treatment , and pre - radiation neurologic symptoms were therefore already present .
limitations of our study include the fact that it was a retrospective , non - randomized analysis with a relatively short follow - up duration and a small number of cases . a single surgeon with considerable experience with craniopharyngioma performed all surgical procedures .
while a randomized controlled study is not practical at this time , a population - based prospective study may provide more reasonable evidence - based treatment guidelines in the future .
our results suggest that if safe resection is possible , surgery should be the treatment of choice to prevent tumor recurrence
. however , if the tumor is located near critical structures or if it recurs , subtotal resection combined with rt or gkrs may be effective both as an initial treatment for patients with a recurrent tumor and as a salvage treatment for recurrent lesions .
 objectivethe aim of this study was to describe a single center 's experience in the management of craniopharyngiomas in children over a 15-year period.methodsthe clinical records of pediatric patients treated for craniopharyngiomas between december 1995 and february 2011 were reviewed . 
 thirty - five pediatric patients diagnosed with craniopharyngioma were treated , and their medical records and imaging data were analyzed retrospectively.resultsthe mean follow - up duration was 76 months ( range , 10 - 195 ) . 
 overall survival and local control rates at 10 years were 94.75.1% and 37.111.9% , respectively . 
 the female - to - male ratio was 16 : 19 , and the mean age was 8.6 years ( range , 1 - 17 ) . 
 initially , gross total resection ( gtr ) was performed in 30 patients ; subtotal resection ( str ) followed by radiotherapy was performed in 5 patients . 
 of the 14 cases that showed recurrence after gtr , 5 patients were treated with gtr , 1 with radiation therapy ( rt ) , 4 with gamma knife radiosurgery ( gkrs ) , and 4 with subtotal resection followed by rt . 
 no patients who underwent rt or gkrs had recurrences . 
 two cases with recurrence after str followed by rt were treated with gtr . 
 one patient died of hormonal insufficiency 64 months after the first surgery . 
 the overall median time progression was 51.2 months ( range , 3 - 182 ) : 49.7 months in the patients who underwent gtr and 60.2 months in the patients who underwent str followed by rt.conclusionif safe resection is possible , gtr at the initial treatment should be attempted to reduce the tumor recurrence . 
 however , if the tumor recurs after the first surgery , rt or gkrs with / without reoperation may be an effective salvage treatment for recurrent craniopharyngioma . 