Jian Gong

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

BACKGROUNDnAdamantinomatous craniopharyngioma (ACP) is a benign but maldevelopmental tumor with a high recurrence rate.nnnOBJECTIVEnTheaim of this study was to investigate the dysregulated biological molecules that play important roles in the recurrence of ACP.nnnMETHODSnWe 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.nnnRESULTSnFour 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.nnnCONCLUSIONSnHigh 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.

Expression of matrix metalloproteinase-9, type IV collagen and vascular endothelial growth factor in adamantinous craniopharyngioma.

To explore the expression of matrix metalloproteinase 9 (MMP-9), type IV collagen (Col IV) and vascular endothelial growth factor (VEGF) in adamantinomatous craniopharyngioma (ACP) and analyze the correlation between the level of these markers and adamantimous craniopharyngiomas recurrence. Expressions of MMP-9, Col IV and VEGF were tested by immunohistochemistry (IHC) in 40 cases of ACP, including 24 cases of primary group and 16 cases of recurred group. The expression level of MMP-9 and VEGF in recurred group were significantly higher than primary group (93.7% vs. 41.7%, Pxa0<xa00.05, 87.5% vs. 45.8%, Pxa0<xa00.05, respectively). The expression of Col IV in the recurred group was significant different from the primary group (Zxa0=xa0−2.619, Pxa0<xa00.05). MMP-9, Col IV and VEGF may be the potential specific bio-marker related to the recurrence of ACP.

Surgical treatment of hypothalamic hamartoma causing central precocious puberty: long-term follow-up

Hypothalamic hamartoma (HH) is a rare condition that often manifests as central precocious puberty (CPP). There is a lack of information available concerning the long-term effectiveness of surgery for the treatment of CPP due to HH. Here the authors describe 3 cases of CPP due to HH, with a follow-up ranging from 9 to 11 years after surgery. Three girls experienced breast growth and menses at 5-18 months of age and 5-36 months of age, respectively. Serum concentrations of luteinizing hormone, follicle-stimulating hormone, and estradiol concentrations ranged from 2.5 to 6.5 mIU/ml, 4.8-5.9 mIU/ml, and 47.9-133.0 pg/ml, respectively. Magnetic resonance imaging confirmed that CPP was caused by HH. Lesions were resected using a right pterional approach. After surgery, endocrine hormone concentrations were normalized, breasts shrunk, and menses ceased in each patient. Moreover, all of them subsequently developed normally and experienced age-appropriate onset of puberty. Each patients height and weight were normal at the most recent follow-up (9-11 years after surgery), and none had experienced learning difficulties. Central precocious puberty due to HH can be successfully treated with resection. In the 3 cases presented, this approach was associated with both short- and long-term efficacy.

Analysis of Survival Prognosis for Children with Symptomatic Optic Pathway Gliomas Who Received Surgery

OBJECTIVEnThe feasibility and prognosis of surgical treatment for children with optic pathway gliomas (OPGs) is controversial. Therefore this study attempted to evaluate the effects of surgery and discuss prognostic factors related to the survival of children with symptomatic OPGs.nnnMATERIALS AND METHODSnOne-hundred twenty-five children diagnosed with OPGs underwent surgery in the Department of Neurosurgery, Beijing Tiantan Hospital from 2003-2016. In this retrospective study, their demographics, clinical characteristics, treatments, and survival outcomes were investigated and summarized.nnnRESULTSnAmong the 125 patients, the 5-year overall survival (OS) rate and progression-free survival (PFS) rate were 84.1% and 70.6%, respectively. In the univariate analysis, patients who received postoperative radiotherapy (RT) after surgery had significantly better 5-year OS and PFS rates than patients who did not receive RT (P < 0.001 for both comparisons), patients who were 3 years old had better PFS rates than younger patients (P < 0.001), and patients with endocrinology symptoms had significantly worse PFS rates than patients with other symptoms (Pxa0=xa00.049). In the multiple regression analysis, postoperative treatment with RT and tumors with a lower pathologic grade were better predictors of OS. An age older than 3 years and postoperative treatment with RT were better predictors of PFS.nnnCONCLUSIONSnSurgery is safe and feasible for children with large volumes of OPGs and symptoms of functional impairment and obstructive hydrocephalus. Furthermore, adjuvant RT after surgery may significantly improve OS and PFS. The pathologic grade is an independent prognostic factor for OS, and the age at diagnosis is an independent prognostic factor for PFS.

Feasibility Analysis for Treatment of Giant Intracranial Benign Tumor by Delayed Operation in Infancy

OBJECTIVEnThe survival rate and prognosis in infants with giant intracranial tumors are significantly worse than in older children. This study aimed to analyze the feasibility of delayed operation for infants with giant intracranial benign tumor by evaluating the initial clinical presentations, expectant treatment measures, perioperative vital signs, and recuperation after surgery.nnnPATIENTS AND DATAnWe reviewed 3 infant patients (average age, 9.33 months; range, 5-12 months) with giant intracranial benign tumors during January 2015 and April 2016. The maximum sections of tumors were 38xa0× 50 mm, 57xa0× 39 mm, and 55xa0× 67 mm, respectively. All clinical presentations, neuroimaging, and laboratory examinations were recorded.nnnRESULTSnObstructive hydrocephalus was observed in 2 infants; ventriculoperitoneal shunts were placed in both before the delayed tumor resection. The disease progressed rapidly in the infant with teratoma and surgery was performed 4 months after placement of the ventriculoperitoneal shunt. The other 2 patients had experienced a 12-month growth and developmental phase and later underwent operations. Gross total resection was achieved in all patients. The pathologic results were consistent with the preoperative diagnosis. During a period of high-quality postoperative care, they remained stable and were discharged without any complications or neurologic deficits, and continued to improve toward their baseline.nnnCONCLUSIONSnDelayed operation enabled infant patients to gain a better physical state, with a stage of full preoperative preparation that may reduce intraoperative/postoperative morbidity and mortality.

Application of computer assisted three-dimensional simulation operation and biomechanics analysis in the treatment of sagittal craniosynostosis

As a surgical method to treat children with sagittal craniosynostosis, calvarial vault reconstruction is subjected to some limitations. In traditional surgical method to treat children with sagittal craniosynostosis, surgical resection and fixation are performed all by the experience of surgical doctor, which is likely to cause individual differences, insecure fixation, configurational asymmetry, and waste of unnecessary fixtures materials. This study aims to provide surgical doctor with objective indicators via 3D simulation combined with biomechanical calculation, so as to improve the surgical efficiency.nnnMETHODSnThe aim of this study is to compare preoperative strategy integrating computer-assisted 3D simulation and biomechanical calculation and traditional strategy. A retrospective method was used to compare the effect and difference between these 2 strategies. The clinical data of 18 patients with sagittal synostosis were collected and compared. Among them, 10 patients were enrolled in Group A applied with traditional treatment method, while 8 were enrolled in Group B applied with preoperative strategy integrating computer-assisted 3D simulation and biomechanical calculation. The aim of this study is to evaluate two treatment methods by investigating indexes between two groups, such as length of operation, blood loss, operation cost, and postoperative complications. Through comparing the cranial index, head circumference, and cranial vault asymmetry index of two groups before and after treatment, the surgical effects of two groups can be evaluated. Moreover, biomechanical analyses for two groups were conducted.nnnRESULTSnRegarding group B, the length of operation was (217±29.3min), blood loss was (70±11.7ml), operation cost was (34,495±8662¥); while for group A, the length of operation was (276±23.5min), blood loss was (90±15.5ml), operation cost was (25,149±4133¥). No postoperative complication was observed for group B, while there was 1 case of central nervous system infection occurred in group A. The cranial indexes of both groups were significantly improved after treatment. The head circumferences of both groups were insignificantly changed after treatment. Through comparing the cranial vault asymmetry index of two groups before and after treatment, the cranial vaults of patients of group A were not so symmetrical as Group B after treatment.nnnCONCLUSIONSnThis research proposes the application of preoperative strategy integrating computer-assisted 3D technique and biomechanical analysis, which allows surgical doctors to have a clear understanding on the surgical process before operation. In particular, this new treatment method for the first time adopts biomechanical calculation to determine the setting position and setting quantity of fixtures materials, so as to realize a firm and symmetrical effect, reduce waste of unnecessary fixtures materials, increase surgical efficiency, reduce length of operation, blood loss, and occurrence of postoperative complications Computer-assisted 3D technique with biomechanical analysis is a new advanced technique for the treatment of sagittal craniosynostosis.

A reformed surgical treatment modality for children with giant cystic craniopharyngioma.

ObjectiveSurgical removal plays an important role in treating children’s craniopharyngioma. For a safe and minimally invasive craniotomy, a reformed surgical modality was proposed in this paper by combining the insertion of an Ommaya reservoir system (ORS) by stereotactic puncture, aspiration of cystic fluid in 2-day interval for consecutive 7–10xa0days, and the delayed tumor resection.Patients and methodsEleven patients (aged from 5 to 9xa0years old) with giant cystic craniopharyngiomas who had undergone the reformed surgical modality during November 2014 and December 2015 were collected as group A. In contrast, seven patients (aged from 5 to 11xa0years old) who had undergone the traditional directed operation without any prior management from January 2014 to October 2014 were collected into group B. A retrospective analysis was performed for both groups at one institution. The preoperative and postoperative clinical presentations, neuroimaging, early postoperative outcome, and the surgery-related complications of both groups were reviewed.ResultsFor group A, the mean value of the maximum tumor diameters shank from 52.36 to 23.82xa0mm after implementing aspiration of the cystic fluid in 1-day interval for consecutive 8.23xa0days. Eight patients (72.73%) in group A underwent a gross total resection (GTR), while two (28.57%) patients underwent GTR in group B. The postoperative electrolyte disturbance rate and endocrine disorder rate of group B were significantly higher than those of group A (42.86 vs 36.36%; 71.43 vs 45.45%). Postoperative long-term diabetes insipidus only occurred in one patient of group B, and postoperative visual deterioration occurred in two patients of group B. Besides, one patient of group B died of severe postoperative hypothalamus dysfunction. Patients with residual tumors were applied with additional adjuvant radiotherapy, and no recurrence was observed in follow-up examinations.ConclusionA favorable outcome can be achieved by combining the insertion of an ORS by stereotactic puncture, aspiration of cystic fluid in 2-day interval for continuously 7–10xa0days, and the delayed tumor resection. This combined treatment modality maybe an effective method to treat children with giant cystic craniopharyngiomas.

Cognitive performance change of pediatric patients after conducting frontal transcortical approach to treat lateral ventricular tumor

ObjectiveApplying frontal transcortical approach to treat lateral ventricular tumor is one of the most common neurosurgical manipulations. The frontal transcortical approach generally passes through the middle frontal gyrus in which there is no major function involved in the traditional sense. However, current researches have suggested that the prefrontal cortex (PFC) plays a central role in the whole network of the brain cognitive frame. In addition, cognitive function is crucial in growing and developmental stages and essential for the educational achievement, especially for children. Based on this, the authors in this study analyzed cognitive performance change of pediatric patients who had accepted frontal transcortical operation in 1-year follow-up and discussed the possibility of higher cognitive functions of the damaged region.Patients and methodsIn this single-center study, 15 pediatric patients (median age at surgery, 9.21xa0years old; range, 6.42–14.17xa0years old) who had been treated with frontal transcortical approach for lateral ventricular tumors were selected as research objects. The cognitive function assessment was conducting by adopting the revised Wechsler Intelligence Scale for Children-fourth edition (WISC-IV). In addition, the resting-state functional magnetic resonance imaging (resting-state fMRI) and diffusion tensor imaging (DTI) were carried out to measure the level of co-activation and to explore the functional connectivity between the brain regions at the preoperative period and 1-year follow-up after surgery.ResultsGTR was achieved in all patients, and all patients were in good condition after surgery. Compared to the preoperative indices of WISC-IV, patients generally had a lower level of indices of the WISC-IV after surgery, for example, the total IQ was declined to Mxa0=xa083.60, SDxa0=xa09.500 from Mxa0=xa095.33, SDxa0=xa013.844 within 1xa0year convalescence. The data of perceptual reasoning (txa0=xa0−xa02.392, pxa0=xa00.016), processing speed (txa0=xa0−xa02.121, pxa0=xa00.033), and total IQ (txa0=xa0−2.638, pxa0=xa00.008) before and after surgery showed statistically significance. Furthermore, decreased functional connectivity and disconnected neural fasciculus were revealed by the size of activation regions in the resting-state fMRI and the reconstruction of three-dimensional images of white matter tracts in the DTI pre- and post-operative.ConclusionThe PFC was not regarded as a major functional area in the past, but the researches at present have shown that the interactions between PFC and other posterior brain regions serve as the basis of the higher cognitive functions. According to imaging manifestations and WISC-IV tasks in this paper, we found that the PFC injury caused by the frontal transcortical approach led to damaged brain structure and impaired the performance of cognitive function. On this basis, we detected that the perceptual reasoning and processing speed maybe have more extensive connections with the middle frontal gyrus.

Retrospective Analysis of the Clinical Characteristics, Therapeutic Aspects, and Prognostic Factors of 18 Cases of Childhood Pineoblastoma

BACKGROUNDnPineoblastoma is a rare malignant tumor of the pineal gland, which is more common in children.nnnMETHODSnWe retrospectively reviewed 18 cases of pineoblastoma in children (10 girls), including general, clinical, and therapeutic information, and factors affecting prognosis.nnnRESULTSnThe median age of the children was 51.7 months (range, 19-156 months). Presenting symptoms included vomiting (64.70%), headache (47.06%), weak or unsteady walking (35.29%), and nausea (29.41%). Rarer symptoms (1 patient each) included limb rigidity, inability to speak, double vision, fever, and Parinaud syndrome. Five and 13 children, respectively, underwent subtotal and gross total resection; 5 and 13 children received adjuvant craniospinal irradiation therapy and chemotherapy. Two children received both craniospinal irradiation and chemotherapy. The 5-year overall survival of the patients was 27.8% (5/18). The survival rate of children older than 4 years (66.7%) was significantly higher than that of younger children (8.3%). The 5-year overall survival rate of boys (50.7%) was higher than that of girls (10.0%); that of children who underwent gross total resection (30.8%) was higher than that of children who underwent subtotal resection (20.0%); and that of children treated with adjuvant craniospinal irradiation (50.7%) was higher than that of those not given craniospinal irradiation (10.0%). However, in each of these 3 comparisons the differences were not significant.nnnCONCLUSIONnPineoblastoma is rare but often fatal, especially in children younger than 4 years. Survival rates tend to be higher in boys, children undergoing gross total resection (rather than subtotal), and those given craniospinal irradiation.

Posterior fossa pilomyxoid astrocytoma with spontaneous hemorrhage in pediatric patients

ObjectThe tumor described in this report was unique because of its involvement with a posterior fossa spontaneous hemorrhage in a pediatric patient; such a case has never been previously described in cases of pilomyxoid astrocytomas and also rarely found in those of pilocytic astrocytomas.MethodsThis report studied a rare case of posterior fossa pilomyxoid astrocytoma (PMA) with critical and dangerous spontaneous hemorrhage. A 7-year-old girl appeared at the outpatient clinic with sudden headache and vomiting.ResultsThe patient underwent gross total tumor resection via suboccipital Medline approach, and no evidence of residual or recurrent tumor was found on magnetic resonance images at two follow-up examinations which were respectively conducted 1 and 5xa0months after resection.ConclusionIn this report, the authors reviewed the literature and discussed the clinical features and treatment of pilomyxoid astrocytoma. It is important to distinguish this tumor variant from the more indolent pilocytic astrocytoma.