Craniopharyngioma In Children Research Articles

Benefits of image-guided stereotactic hypofractionated radiation therapy as adjuvant treatment of craniopharyngiomas. A review.

Craniopharyngiomas account for 5.6-13% of intracranial tumors in children. Despite being histologically benign, these tumors remain a major neurosurgical challenge because of the typical tight adherence to adjacent critical structures. The optimal therapeutic approach for this disease is controversial. Large cystic size and adherence to neurovascular, neuroendocrine, and optic structures without a clear line of cleavage make complete resection problematic and often hazardous. For these reasons, partial resection and adjuvant treatment play an important role. Post-operative radiation therapy (RT) following either complete or incomplete tumor removal is associated with significantly decreased recurrence rates. The aim of this review is to analyze the potential advantage of the most modern technical advancements for RT of craniopharyngiomas. This narrative review on the topic of craniopharyngiomas was based on published data available on PUBMED/Medline. All data concerning adjuvant or upfront radiation therapy treatment of craniopharyngioma were reviewed and summarized. A more detailed analysis of fractionated frameless steretactic radiosurgery of these tumors is provided as well. We reviewed the possible improvement provided by intensity modulated beams, arc therapy, image guidance, proton radiation, and fractionated stereotactic radiosurgery. Many published findings on outcome and toxicity after RT involve the use of relatively outdated RT techniques. Technologic improvements in imaging, radiation planning, and delivery have improved the distribution of radiation doses to desired target volumes and reduced the dose to nearby critical normal tissues. Currently available techniques, providing image guidance and improved radiation doses distribution profile, have shown to maintain the efficacy of conventional techniques while significantly reducing the toxicity. Image-guided radiosurgery holds the dose distributions and precision of frame-based techniques with the remarkable advantage of multiple-session treatments that are better tolerated by sensitive peritumoral structures, such as the optic pathway and hypothalamus. This, together with the comfort of a frameless technique, candidates frameless image-guided radiosurgery to be the first option for the adjuvant post-operative treatment of craniopharyngiomas in children and young adults when total resection cannot be achieved, in particular those with hypothalamic involvement, and when the residual tumor is mostly solid.

A Consideration of Potential Modifications in Current Grading Scales of Craniopharyngioma in Children

A Consideration of Potential Modifications in Current Grading Scales of Craniopharyngioma in Children

Long-term visual outcomes of craniopharyngioma in children.

Visual function is a critical factor in the diagnosis, monitoring, and prognosis of craniopharyngiomas in children. The aim of this study was to report the long-term visual outcomes in a cohort of pediatric patients with craniopharyngioma. The study design is a retrospective chart review of craniopharyngioma patients from a single tertiary-care pediatric hospital. 59 patients were included in the study. Mean age at presentation was 9.4years old (range 0.7-18.0years old). The most common presenting features were headache (76%), nausea/vomiting (32%), and vision loss (31%). Median follow-up was 5.2years (range 1.0-17.2years). During follow-up, visual decline occurred in 17 patients (29%). On Kaplan Meier survival analysis, 47% of the cases of visual decline occurred within 4months of diagnosis, with the remaining cases occurring sporadically during follow-up (up to 8years after diagnosis). In terms of risk factors, younger age at diagnosis, optic nerve edema at presentation, and tumor recurrence were found to have statistically significant associations with visual decline. At final follow-up, 58% of the patients had visual impairment in at least one eye but only 10% were legally blind in both eyes (visual acuity 20/200 or worse or <20° of visual field). Vision loss is a common presenting symptom of craniopharyngiomas in children. After diagnosis, monitoring vision is important as about 30% of patients will experience significant visual decline. Long-term vision loss occurs in the majority of patients, but severe binocular visual impairment is uncommon.

The clinical characteristics and surgical treatment of infradiaphragmatic craniopharyngioma in children

Objective To analyze the clinical characteristics and surgical treatment strategy of infradiaphragmatic craniopharyngiomas in children. Methods A retrospective analysis was conducted on 68 cases of childhood-onset infradiaphragmatic craniopharyngiomas who underwent surgical treatment at Department of Neurosurgery, Nanfang Hospital of Southern Medical University from December 2000 to January 2015. Among them, 19 patients received transsphenoidal surgery, 49 cases underwent craniotomy including 12 through lateral approach and 37 through anterior interhemispheric fissure approach. All patients were clinically followed up post operation. Analysis was conducted to explore the factors affecting the surgical operations, approaches and recurrence of infradiaphragmatic craniopharyngiomas. Results Infradiaphragmatic craniopharyngioma was common in childhood-onset craniopharyngiomas and accounted for 41.7%(68/163). Growth retardation and impaired vision were two most common clinical manifestations which accounted for 86.8%(59/68) and 69.1%(47/68), respectively. There were 58.8%(40/68) of the patients who presented pre-operative panhypopituitarism. The follow-up ranged from 18 to 270 months (mean: 71.4±53.4 month), and 13 out of 68 (19.1%) patients showed recurrence during the follow-up, 20 out of 28 (71.4%) non-panhypopituitarism patients presented newly onset deficiency of pituitary hormones. Multiple factors, including the tumor size bigger than 10 cm3, ratio of suprasellar tumor diameter to intrasellar tumor diameter >2, sagittal maximal tumor diameter greater than 4 cm, tumor with suprasellar complex tumor cysts, were more likely to result in craniotomy (all P<0.05). Tumor resection not along the membrane boundary of intrasellar part and the maximal diameter larger than 2.5 cm were two independent risk factors for recurrence (all P<0.05). Conclusions Infradiaphragmatic craniopharyngioma have negative impacts upon the patients’ quality of life. Exposure and removal of the intrasellar part of tumor could reduce the risk of recurrence for either transsphenoidal operation or craniotomy. Anterior interhemispheric fissure approach seems to be helpful for the exposure of intrasellar tumor and superior to the lateral approach. Key words: Craniopharyngioma; Neoplasm recurrence, local; Infradiaphragmatic; Child; Surgical approach; Panhypopituitarism

Outcomes of microsurgical resection of recurrent craniopharyngiomas in children

Objective To investigate the clinical outcomes in children with recurrent craniopharyngimas after microsurgical resection of the tumors. Methods The clinical outcomes of 73 cases of recurrent childhood craniopharygngiomas from April 2008 to December 2014 were retrospectively analyzed. All patients underwent microsurgical resection of the tumors at Neurosurgery Department of Sanbo Brain Hospital, Capital Medical University. Results Total removal of the tumor was achieved in 64 (87.7%) of the 73 patients and the subtotal resection rate was 12.3% (9 cases). The perioperative mortality was 2.7% (2 cases). A total of 52 patients were followed up from 6 to 93months, with the median of 45 months. The tumor recurrence rate was 36.5% (19/52) during the follow-up period. For those patients whose tumors were removed totally, the tumor recurrence rate was 30.2% (13/43), and it was 66.7% (6/9) for those whose tumors were removed subtotally. There was statistical difference between the two groups (P=0.039). The fatality rate was 13.4% (7/52) during the follow-up. We used the Karnofsky Performance Scale to assess the life quality of the 45 children. The results showed that 4 children scored 100 points, 35 scored 90, 3 scored 80, 2 scored 60, and 1 scored 40. There were 26 (50.0%, 26/52) children demonstrating significant growth retardation, 24 (46.2%, 24/52) needed oral desmopressin to treat diabetes insipidus, and 5 (9.6%, 5/52) showed significant obesity. Conclusions Microsurgical resection of recurrent craniopharyngiomas in children may result in good outcomes. The treatment of postoperative hypothalamic-pituitary deficiency is still a challenge. Key words: Craniopharyngioma; Recurrence; Microsurgery; Child

Effect of Preserving the Pituitary Stalk During Resection of Craniopharyngioma in Children on the Diabetes Insipidus and Relapse Rates and Long-Term Outcomes.

The objective of this study was to investigate the effect of preserving an infiltrated pituitary stalk during the resection of craniopharyngioma of pituitary stalk origin on postoperative outcomes and thus provide a theoretical basis for microsurgical treatment and prognosis. We screened the clinical data of all 103 pediatric patients with craniopharyngioma undergoing surgical treatment at our department between January 2006 and January 2013 and conducted a retrospective analysis of 82 patients with craniopharyngioma originating in the pituitary stalk. The patients were followed up from 12 months to 8 years. We analyzed the effect of preserving the pituitary stalk on the early and persistent diabetes insipidus rates, postoperative relapse rate, and mortality. In the total resection group (n = 67), the early and persistent diabetes insipidus rates were significantly lower in the 46 patients (68.7%) with a pituitary stalk than in those whose pituitary stalk was removed (P < 0.05); no significant difference was observed in the relapse rate between the 2 subgroups (P > 0.05). In the subtotal resection group (n = 15), a significant difference was observed in the early and persistent diabetes insipidus rates (P < 0.05), but no significant difference was observed in the relapse rate between the patients with a pituitary stalk and those whose pituitary stalk was removed (P > 0.05). For children with craniopharyngioma of pituitary stalk origin, preserving the pituitary stalk has a significant effect on the early and persistent diabetes insipidus rates. When intraoperative exploration showed excessive adhesion between the tumor and pituitary stalk, we opted to preserve the pituitary stalk, which significantly reduced the early and persistent postoperative diabetes insipidus rates, without significantly increasing the relapse or mortality rate.

Long-term visual outcomes of craniopharyngioma in children

Long-term visual outcomes of craniopharyngioma in children

Review of management and morbidity of pediatric craniopharyngioma patients in a low-middle-income country: a 12-year experience.

Management of craniopharyngioma in children is challenging, and their quality of life can be significantly affected. Series describing this from low-middle income countries (LMIC) are few. The study provides a retrospective chart review of pediatric patients <18years old, diagnosed with craniopharyngioma between 2003 and 2014, and treated at King Hussein Cancer Center, Jordan. Twenty-four patients (12 males) were identified. Median age at diagnosis was 7.4years (0.9-16.4years). Commonest symptoms were visual impairment and headache (71%). Review of seventeen preoperative MRIs showed hypothalamic involvement in 88% and hydrocephalus in 76%. Thirteen patients (54%) had multiple surgical interventions. Five patients (21%) had initial gross total resection. Eleven patients (46%) received radiotherapy and six (25%) intra-cystic interferon. Five years' survival was 87±7% with a median follow-up of 4.5years (0.3-12.3years). Four patients (17%) died; one after post-operative cerebral infarction and three secondary to hypothalamic damage. At their last evaluation, all but one patient required multiple hormonal supplements. Ten patients (42%) had best eye visual acuity (VA) >20/40, and four (16%) were legally blind. Eleven patients (46%) were overweight/obese; one had gastric bypass surgery. Seven patients had hyperlipidemia, and eight developed fatty liver infiltration. Eleven patients (65%) were attending schools and one at college. Nine of the living patients (53%) expressed difficulty to engage in the community. Management of pediatric craniopharyngioma is particularly complex and demanding in LMIC. Multidisciplinary care is integral to optimize the care and minimize the morbidities. A management outline for LMIC is proposed.

Endocrine Disorders Developing after Surgical Intervention of Craniopharyngioma in Children.

Craniopharyngiomas, albeit their benign nature, can cause severe damage to visual, hypothalamic, endocrine and neurologic functions which make their total resection an inevitable approach to save the patient's life. However, significant therapy-related long term complications make those traditional treatment options debatable and hazardous. This review will focus on the various complications that affect the childrens' quality of life considerably such as, diabetes insipidus, precocious puberty and hypothalamic obesity.

Endocrine Disorders Developing after Surgical Intervention of Craniopharyngioma in Children.

Craniopharyngiomas, albeit their benign nature, can cause severe damage to visual, hypothalamic, endocrine and neurologic functions which make their total resection an inevitable approach to save the patient's life. However, significant therapy-related long term complications make those traditional treatment options debatable and hazardous. This review will focus on the various complications that affect the childrens' quality of life considerably such as, diabetes insipidus, precocious puberty and hypothalamic obesity.

Anaesthetic management of children with craniopharyngioma

AbstractThe perioperative management of craniopharyngioma in children is quite challenging not only to the neurosurgeons and anaesthesiologists but also to the oncologists, endocrinologists and intensivists. The various concerns include size of the tumour and its effects on intracranial pressure, vicinity of the tumour to nearby vessels and neural structures, endocrine disturbances, compressive symptoms, paediatric age, obesity-related problems, high recurrence rate of the tumour, undesired effects of radiotherapy, high morbidity and mortality. As anaesthesiologists, we deal with most of the challenges. The success of the outcome depends on the proper identification of the patient, pre-operative optimisation, surgical decision, awareness of the complications, timely intervention and optimal post-operative care.

Anesthetic Considerations for Craniopharyngioma Resection in Pediatric Patient with Fontan Physiology: A Case Report

Fontan procedure is a palliative surgery done for patients born with single ventricle physiology. An understanding of the hemodynamic alterations in such a patient is important for successful perioperative management. Added to this challenge is the Craniopharyngioma which constitute about 2-6% of all the intracranial tumors in pediatric age group. Management of craniopharyngiomas in children is very challenging for anesthesiologist, owing to the developing neurological and physiological status, handling of a growing brain, perioperative endocrinological complications, and the management of hydration. We discuss the anesthetic considerations for a 6 years old female with Fontan circulation going for craniopharyngioma resection under general anesthesia.

Intracystic bleomycin for cystic craniopharyngiomas in children.

Craniopharyngiomas are the most common benign histological tumours to involve the hypothalamo-pituitary region in childhood. Cystic craniopharyngiomas account for more than 90% of the tumours. The optimal treatment of cystic craniopharyngioma remains controversial. Radical resection is the treatment of choice in patients with favourable tumour localisation. When the tumour localisation is unfavourable, a gross-total or partial resection followed by radiotherapy is the main treatment option in adults. However, it presents a risk of morbidity, especially for children. Intracystic bleomycin has been utilised potentially to delay the use of radiotherapy or radical resection, to decrease morbidity. This review is the second update of a previously published Cochrane review. To assess the benefits and harmful effects of intracystic bleomycin in children from birth to 18 years with cystic craniopharyngioma when compared to placebo (no treatment), surgical treatment (with or without adjuvant radiotherapy) or other intracystic treatments. We searched the electronic databases CENTRAL (2016, Issue 1), MEDLINE/PubMed (from 1966 to February 2016) and EMBASE/Ovid (from 1980 to February 2016) with pre-specified terms. In addition, we searched the reference lists of relevant articles and reviews, conference proceedings (International Society for Paediatric Oncology 2005-2015) and ongoing trial databases (Register of the National Institute of Health and International Standard Randomised Controlled Trial Number (ISRCTN) register) in February 2016. Randomised controlled trials (RCTs), quasi-randomised trials or controlled clinical trials (CCTs) comparing intracystic bleomycin and other treatments for cystic craniopharyngiomas in children (from birth to 18 years). Two review authors independently performed the study selection, data extraction and 'Risk of bias' assessment. We used risk ratio (RR) for binary data and mean difference (MD) for continuous data. If one of the treatment groups experienced no events and there was only one study available for the outcome, we used the Fischer's exact test. We performed analysis according to the guidelines in the Cochrane Handbook for Systematic reviews of Interventions. We could not identify any studies in which the only difference between the treatment groups was the use of intracystic bleomycin. We did identify a RCT comparing intracystic bleomycin with intracystic phosphorus(32) ((32)P) (seven children). In this update we identified no additional studies. The included study had a high risk of bias. Survival could not be evaluated. There was no clear evidence of a difference between the treatment groups in cyst reduction (MD -0.15, 95% confidence interval (CI) -0.69 to 0.39, P value = 0.59, very low quality of evidence), neurological status (Fisher's exact P value = 0.429, very low quality of evidence), third nerve paralysis (Fischer's exact P value = 1.00, very low quality of evidence), fever (RR 2.92, 95% CI 0.73 to 11.70, P value = 0.13, very low quality of evidence) or total adverse effects (RR 1.75, 95% CI 0.68 to 4.53, P value = 0.25, very low quality of evidence). There was a significant difference in favour of the (32)P group for the occurrence of headache and vomiting (Fischer's exact P value = 0.029, very low quality of evidence for both outcomes). Since we identified no RCTs, quasi-randomised trials or CCTs of the treatment of cystic craniopharyngiomas in children in which only the use of intracystic bleomycin differed between the treatment groups, no definitive conclusions could be made about the effects of intracystic bleomycin in these patients. Only one low-power RCT comparing intracystic bleomycin with intracystic (32)P treatment was available, but no definitive conclusions can be made about the effectiveness of these agents in children with cystic craniopharyngiomas. Based on the currently available evidence, we are not able to give recommendations for the use of intracystic bleomycin in the treatment of cystic craniopharyngiomas in children. High-quality RCTs are needed.

Influence of previous treatments on repeat surgery for recurrent craniopharyngiomas in children.

Craniopharyngioma is a common pediatric brain tumor, with a high rate of recurrence after primary treatment. This retrospective study investigated the effect of various primary treatments on surgical strategies and outcomes for recurrent craniopharyngiomas. The study population comprised 35 children (mean age 8.77years, range 1-16years) with recurrent craniopharyngioma re-operated from January 1990 to January 2009. The recurrent craniopharyngiomas were excised whenever possible. For analysis, the patients were divided into four groups according to the primary treatment: radical tumor resection (A), incomplete tumor resection (B), radiotherapy + incomplete tumor resection (C), and Ommaya reservoir placement + incomplete tumor resection (D). Group B had a significantly shorter recurrence-free interval than groups A, C, or D. Outcomes were significantly different among the four groups. The hypothalamic status scores of groups A (2.38±0.27) and C (2.28±0.42) were significantly higher than that of group B (1.64±0.20). There were no statistical differences between any two other groups. In children, the primary treatments for craniopharyngioma should be considered when choosing the surgical strategy for recurrence. Radiotherapy before repeated surgery can result in a worse functional outcome and hypothalamic-pituitary function.

Craniopharyngiomas : Radiological Differentiation of Two Types.

ObjectiveTo determine imaging features that may separate adamantinomatous and papillary variants of craniopharyngiomas given that tumors with adamantinomatous signature features are associated with higher recurrence rates, morbidity, and mortality. We specifically reviewed calcification on CT, T1 bright signal intensity, and cystic change on T2 weighted images for differentiating these two types.MethodsWe retrospectively reviewed the MRI and CT studies in 38 consecutive patients with pathologically proven craniopharyngiomas between January 2004 and February 2014 for the presence of calcification on CT scans, bright signal intensity on T1 weighted images, and cystic change on T2 weighted images.ResultsOf the 38 craniopharyngiomas, 30 were adamantinomatous type and 8 were papillary type. On CT scans, calcification was present in 25 of 38 tumors. All calcified tumors were adamantinomatous type. Twenty four of 38 tumors had bright signal intensity on T1 weighted images. Of these 24 tumors, 22 (91.7%) were adamantinomatous and 2 were papillary type. Cystic change on T2 weighted images was noted in 37 of 38 tumors; only 1 tumor with papillary type did not show cystic change.ConclusionT1 bright signal intensity and calcification on CT scans uniformly favor the adamantinomatous type over papillary type of craniopharyngioma in children. However, these findings are more variable in adults where calcification and T1 bright signal intensity occur in 70.6% and 58.8% respectively of adult adamantinomatous types of craniopharyngiomas.

Giant craniopharyngiomas in children: short- and long-term implications.

Craniopharyngiomas (CPs) are histologically benign tumors, yet they may carry an unfavorable prognosis. "Giant" tumors are associated with worse prognosis. Our aim was to evaluate the impact of tumor's size on different short- and long-term clinical factors. Between 2002 and 2012, a total of 36 consecutive CP patients less than 18years of age and with at least 12months of post-operative follow-up (FU) underwent a total of 54 operations for excision of CPs. Gross total resection (GTR) was the goal for all the first surgical resections. Twenty-seven patients were identified as eligible for inclusion in this study. Data were retrospectively collected by reviewing pertinent clinic/office notes and inpatient records as well as pre- and post-operative imaging. Long-term neurosurgical, ophthalmological, and endocrinological outcomes were obtained from records of the most recent FU office visit. Statistical analysis was performed to compare data from patients with tumors greater than or equal to 4.5cm (nine patients) to those with smaller ones (<4.5cm; 18 patients). Mean age at the time of surgery was 5.4years (median 5years, range 1.3-15.3years) for patients in the large-tumor group (LTG) and 8.9years (median 9.6years, range 2.1-17.1years) for the small-tumor group (STG). Average duration of follow-up was 82.1 and 105.4months for LTG and STG patients, respectively. There was a noticeable difference in the rate of emergent surgeries between the two groups (33.3 vs. 5.5% in the LTG and STG, respectively; p = 0.055) as well as in recurrent surgeries (RR = 3.76; CI = 95%, 1.793-7.877) and radiotherapy (RR = 2, 95% CI 0.775-5.154). Rates of residual tumor on both initial post-operative imaging and last FU imaging were significantly increased in LTG patients (44.5 vs. 27.7% and 66.6 vs. 16.6%; respectively). Progression-free survivals (PFS) assessed at 2 and 5years were 33.3 vs. 73.3% (RR = 2.2, 95% CI = 0.171-1.202) and 33.3 vs. 53.3% (RR = 1.6, 95% CI 0.221-1.765) in favor of smaller tumors. No difference in 2-, 5-, and 10-year overall survival was found. We found no significant difference in mean BMI at last follow-up between the two groups (23.83 ± 4.86 and 27.33 ± 8.09, respectively; p = 0.27), although significantly more patients in the LTG had shorter stature (mean height SDS -1.72 ± 1.88 and -0.17 ± 1.08 in LTG and STG patients, respectively; p = 0.027). Tumor's size has significant impact on the management of CP in children. It affects both short-term factors (initial symptoms, urgency of surgical resection, extent of resection, and perioperative morbidity) as well as long-term parameters (PFS, rate of adjuvant treatments/recurrent surgeries, and metabolic/endocrinal/ophthalmological and functional outcomes). We think that a proper, world-wide accepted definition of what is "large," "giant," or even "monstrous" CP should be established. This will enable carrying multi-institutional studies on a larger group of patients, allowing further determining the importance of tumor's size in the management and outcome of craniopharyngiomas in children.

Stereotactic intracavitary brachytherapy with P-32 for cystic craniopharyngiomas in children

Although microsurgery remains the first-line treatment, gross total resection of cystic craniopharyngeomas (CP) is associated with significant morbidity and mortality and the addition of external irradiation to subtotal resection proves to achieve similar tumor control. However, concern regarding long-term morbidity associated with external irradiation in children still remains. With this retrospective analysis, the authors emphasize intracavitary brachytherapy using phosphorus-32 (P-32) as a treatment option for children with cystic CP. Between 1992 and 2009, 17 children (median age 15.4 years; range 7-18 years) with cystic CP underwent intracavitary brachytherapy using P-32. Eleven patients were treated for recurrent tumor cysts; 6 patients were treated primarily. MR imaging revealed solitary cysts in 7 patients; 10 patients had mixed solid-cystic lesions (median tumor volume 11.1ml; range 0.5-78.9ml). The median follow-up time was 61.9 months (range 16.9-196.6 months). Local cyst control could be achieved in 14 patients (82 %). Three patients showed progression of the treated cystic formation (in-field progression) after a median time of 8.3 months (range 5.3-10.3 months), which led to subsequent interventions. The development of new, defined cysts and progression of solid tumor parts (out-of-field progression) occurred in 5 patients and led to additional interventions in 4 cases. There was neither surgery-related permanent morbidity nor mortality in this study. The overall progression-free survival was 75, 63, and 52 % after 1, 3, and 5 years, respectively. Intracavitary brachytherapy using P-32 represents a safe and effective treatment option for children harboring cystic CP, even as primary treatment. However, P-32 does not clearly affect growth of solid tumor parts or the development of new cystic formations.

The challenges in anesthetic management of pediatric craniopharyngioma

Craniopharyngiomas constitute about 2–6% of all the intracranial tumors in the pediatric age group. Management of craniopharyngiomas in children is challenging for not only surgeons and endocrinologists but for anesthesiologists as well, owing to the developing neurological and physiological status, handling of a growing brain, perioperative endocrinological complications, and the management of hydration. We report a case of a 4-year-old child who had presented to our hospital with progressive loss of vision over a period of 2 months.

Intracystic bleomycin for cystic craniopharyngiomas in children.

Craniopharyngiomas are the commonest benign histological tumours to involve the hypothalamo-pituitary region in childhood. Cystic craniopharyngiomas comprise more than 90% of the tumours. The optimal treatment of cystic craniopharyngioma remains controversial. Radical resection is the treatment of choice in patients with favourable tumour localisation. When the tumour localisation is unfavourable, a gross-total or partial resection followed by radiotherapy is the main treatment option in adults. However, it presents a risk of morbidity, especially for children. Intracystic bleomycin has been utilised potentially to delay the use of radiotherapy or radical resection, to decrease morbidity. This review is an update of a previously published Cochrane review. To assess the benefits and harmful effects of intracystic bleomycin in children from birth to 18 years with cystic craniopharyngioma when compared to placebo (no treatment), surgical treatment (with or without adjuvant radiotherapy) or some other intracyctic treatments. We searched the electronic databases CENTRAL (2014, Issue 1), MEDLINE/PubMed (from 1966 to March 2014) and EMBASE/Ovid (from 1980 to March 2014) with pre-specified terms. In addition, we searched the reference lists of relevant articles and reviews, conference proceedings (International Society for Paediatric Oncology 2005-2013) and ongoing trial databases (Register of the National Institute of Health and International Standard Randomised Controlled Trial Number (ISRCTN) register) in May 2014. Randomised controlled trials (RCTs), quasi-randomised trials or controlled clinical trials (CCTs) comparing intracystic bleomycin and other treatments for cystic craniopharyngiomas in children (from birth to 18 years). Two review authors independently performed the data extraction and 'Risk of bias' assessment. We used risk ratio (RR) for binary data and mean difference (MD) for continuous data. We planned that if one of the treatment groups experienced no events and there was only one study available for the outcome, we would use the Fischer's exact test. We could not identify any studies in which the only difference between the treatment groups was the use of intracystic bleomycin. We did identify a RCT comparing intracystic bleomycin with intracystic phosphorus(32) ((32)P) (n = 7 children). The trial had a high risk of bias. Survival could not be evaluated. There was no evidence of a significant difference between the treatment groups in cyst reduction (MD -0.15, 95% confidence interval (CI) -0.69 to 0.39, P value = 0.59), neurological status (Fisher's exact P value = 0.429), 3rd nerve paralysis (Fischer's exact P value = 1.00), fever (RR 2.92, 95% CI 0.73 to 11.70, P value = 0.13) or total adverse effects (RR 1.75, 95% CI 0.68 to 4.53, P value = 0.25). There was a significant difference in favour of the (32)P group for the occurrence of headache and vomiting (Fischer's exact P value = 0.029 for both outcomes). Since we identified no RCTs, quasi-randomised trials or CCTs of the treatment of cystic craniopharyngiomas in children in which only the use of intracystic bleomycin differed between the treatment groups, no definitive conclusions could be made about the effects of intracystic bleomycin in these patients. Only one low-power RCT comparing intracystic bleomycin with intracystic (32)P treatment was available, but no definitive conclusions can be made about the effectiveness of these agents in children with cystic craniopharyngiomas. Based on the currently available evidence, we are not able to give recommendations for the use of intracystic bleomycin in the treatment of cystic craniopharyngiomas in children. High-quality RCTs are needed.

High expression levels of CXCL12 and CXCR4 predict recurrence of adamanti-nomatous craniopharyngiomas in children.

Adamantinomatous craniopharyngioma (ACP) is a benign but maldevelopmental tumor with a high recurrence rate. Theaim of this study was to investigate the dysregulated biological molecules that play important roles in the recurrence of ACP. We first performed microarray analysis on tumor samples from two pediatric patients with recurrent ACP and from two pediatric ACP patients without recurrence after a one-year follow-up. The expression of CXCL12 and CXCR4 in 45 specimens of pediatric ACP was further evaluated by immunohistochemistry. These results were correlated with the clinicopathological parameters and survival of the patients. Four downregulated genes (APC, ITGA, MCAM, and TIMP4) and 16 upregulated genes (CST7, CTSK, CTSL1, CXCL12, CXCR4, FN1, FXYD5, ITGB3, MMP2, MMP3, MMP7, MMP9, NR4A3, PLAUR, TIMP2, and VEGFA) were found in the recurrent patients. CXCL12 and CXCR4 were highly expressed in 13 patients (28.9%) and 14 patients (31.1%), respectively. High levels of CXCL12 and CXCR4 expression were significantly associated with a poor recurrence-free survival and were the prognostic factors for ACP recurrence in pediatric patients. High levels of CXCL12 and CXCR4 expression were associated with ACP recurrence. The role of CXCL12 and CXCR4 in the development of brain tumors requires further research.