Treatment Outcomes of Pediatric Craniopharyngioma : A 15-Year Retrospective Review of 35 Cases

Objective

The aim of this study was to describe a single center''s experience in the management of craniopharyngiomas in children over a 15-year period.

Methods

The 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.

Results

The mean follow-up duration was 76 months (range, 10-195). Overall survival and local control rates at 10 years were 94.7±5.1% and 37.1±11.9%, 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.

Conclusion

If 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.     

A systematic review of the results of surgery and radiotherapy on tumor control for pediatric craniopharyngioma

Objective

Craniopharyngiomas are rare tumors with bimodal incidence in the pediatric and adult age groups. Treatment strategies range from aggressive resection to planned limited resection combined with adjuvant therapies. Currently there is no consensus for standard of care for pediatric craniopharyngioma.

Materials and methods

We performed a systematic review of the published literature on pediatric craniopharyngioma. Patients were grouped based on extent of resection into gross total resection (GTR), subtotal resection (STR), and biopsy procedures. These groups were compared with respect to tumor control. Chi square was used to compare rates of recurrence. Kaplan–Meier was used to generate progression-free survival (PFS) estimates. Cox proportional hazard modeling was used to evaluate risk of progression. Each extent of resection group was also subdivided based on adjuvant therapy and compared.

Results

A total of 109 studies described extent of resection resulting in a cohort of 531 patients. Recurrence data were available for 377 patients. There was no difference in 1- or 5-year PFS between the groups who underwent GTR and STR combined with radiation (XRT; log-rank; p?=?0.76; 1-year PFS 89 vs 84 %; 5-year PFS 77 vs 73 %, respectively). One-year PFS was 84 % for STR+XRT compared to 76 % for STR alone while 5-year PFS was 73 % for STR+XRT compared to 43 % for STR alone (log-rank; p?=?0.003).

Conclusion

Although there are limitations of a systematic review of retrospective data, our results suggest that STR+XRT of pediatric craniopharyngioma is associated with similar rates of tumor control as GTR.     

Radical resection of craniopharyngioma

Introduction The best management of craniopharyngioma in children remains a controversial topic among neurosurgeons. The two treatments for craniopharyngioma most commonly discussed in the literature are primary total resection and limited resection followed by radiotherapy. Without ignoring the challenging behavior of these tumors, we strongly believe that the first approach in a child with a craniopharyngioma is to attempt total removal. Trying to remove a craniopharyngioma that has been treated previously with other methods is, in our experience, much more dangerous because of adherences of the tumor to vascular and neural structures.Material and methods Between 1988 and 2004, we operated on 153 patients with craniapharyngioma (40% female and 60% male), whose ages at the time of surgery ranged from 15 days to 21 years (mean 10.5 years). Eighty-seven percent of the patients were found to have some visual disturbance and 42% endocrinological alterations. Fifty-four percent of the patients presented hydrocephalus, but only 18% had shunting. Gross total removal was attempted in all patients. Among the 153 patients, the tumor was prechiasmatic in 35 and retrochiasmatic in 112; in ten, these were considered giant forms, and eight had a posterior fossa extension. We performed 84 single and 69 combined approaches.Results We achieved total removal in 69% of our patients. None of our patients regarded as having undergone total tumor resection disclosed recurrence after a follow-up of 1–16 years. Radiation therapy was administered in children with subtotal removal. All children underwent total removal, but only 62% of those who underwent subtotal removal had good outcomes. After surgery, endocrinological status worsened in almost all patients, but visual status improved markedly.Conclusions The treatment of choice in craniopharyngioma in childhood is total resection in order to avoid radiation therapy and recurrence. When total resection is not possible, subtotal resection plus radiation therapy is the alternative.     

Outcomes and failure patterns in childhood craniopharyngiomas

Past studies of craniopharyngiomas in children have shown overall survival (OS) up to 95% at 5 years and 80% progression-free survival (PFS) at 5 years, although many of these series predate modern neuroimaging and current therapeutic management. Moreover, little mention has been made of failure patterns for craniopharyngioma in children. To obtain a contemporary assessment of outcome among pediatric craniopharyngioma patients, and also to determine the failure patterns for this tumor, we completed a retrospective study of a consecutive cohort of all children with craniopharyngioma diagnosed at the Johns Hopkins Hospital from 1980 to 1996. Resection was performed in 30 children, in 8 of whom gross total resection (GTR) was achieved. Initial treatment took the form of GTR followed by observation for 8, subtotal resection (STR) plus observation in 11, and STR followed immediately by radiotherapy in 8. The timing of radiotherapy following STR was unclear for 3. OS was 95.2% (SE= 4.7%) at 5 years, with only 2 children dying after 4 years from diagnosis. Five-year PFS was 59.4% (SE=10.2%). Before surgery, 19 children had visual loss and 15, endocrine deficits; after surgery, 21 children had visual loss and 29, endocrine deficits. Median time to relapse was 0.98 years (SD=2.5 years). Radiographic (n=4) and clinical (n=7) relapses did not differ in time to progression (P=0.32), but radiographic relapses were significantly associated with age at diagnosis less than 5 years (P=0.02). Degree of resection was not significantly associated with PFS (P=0.32) or with postoperative visual or endocrine deficits. Absence of calcification on diagnostic neuroimaging (n=8) was significantly associated with improved PFS [5-year PFS 100% vs. 42.9% (SE=14.7%), P=0.02], even when adjusted for extent of resection (P=0.03). Preoperative visual loss was predictive of postoperative visual loss (P=0.03). Survival for children diagnosed with craniopharyngioma in the current era is outstanding, even with relapse, although postoperative visual and endocrinological morbidities are high. Failures occurred both radiographically and clinically, typically in the first 3–4 years after surgery, suggesting a need for close surveillance initially with neuroimaging, particularly in younger children, and also clinical examination. The short times to relapse observed here may stem from a tendency to delay radiotherapy until recurrence. Lack of calcification at diagnosis is associated with a tendency to remain free of relapse. Received: 10 May 1998     

Endonasal endoscopic resection of suprasellar craniopharyngioma: A retrospective single-center case series

IntroductionEndoscopic endonasal approach (EEA) has recently been proposed as an option for resection of primary and recurrent suprasellar craniopharyngioma. However, surgical outcome has not yet been fully evaluated, especially in regards to recurrent cases.MethodsWe analysed our institution (Sir Charles Gairdner University Hospital, Perth, Australia) case-series retrospectively. There were 16 patients operated through an endonasal endoscopic approach from February 2014 to February 2019 for suprasellar craniopharyngiomas. There were 14 primary, and two recurrent lesions. Extent of resection, complications, visual and endocrinological outcomes are presented.ResultsMean age of the patients was 42.9 ± 19.3 years old, with 56% female. The most common clinical symptoms were headaches (9 patients, 56%) and bi-temporal hemianopsia (9 patients, 56%), followed by unilateral optic neuropathy (5 cases, 31%), memory loss (1 case, 6%), hydrocephalus (1 case, 6%), delayed growth and puberty (1 case, 6%), and secondary amenorrhoea (1 case, 6%). Only two cases (12%) initially presented with normal visual function. Gross total resection (GTR) was achieved in 10/16 patients (62.5%), with subtotal resection (STR) in the remainder. Visual symptoms improved in 13/16 patients (81%) and remained unchanged in 3/16 patients (19%). Most common complications included new endocrinological deficit in nine patients (56%), mostly diabetes insipidus, and cerebrospinal fluid leak requiring a new intervention in three patients (19%). There was one mortality case (complicated meningitis, stroke and vasospasm). Mean follow-up time was 22.05 ± 14 months and three patients (19%) had a recurrence of the disease during this period and were referred for radiation therapy.ConclusionEndonasal endoscopic approach is a safe and effective surgical option for both primary and recurrent suprasellar craniopharyngiomas.     

Clinicopathological features of myxopapillary ependymoma

Myxopapillary ependymoma (MPE) is a rare and distinct variant of ependymoma with a tendency for local recurrence and metastasis. Its clinicopathological spectrum is heterogenous, underscoring the need to understand and characterize MPE for better diagnosis and treatment. The purpose of this study was to explore the tumor biology and assess the management of patients with MPE. Tumors from a cohort of 19 patients were analyzed by light microscopy, electron microscopy, immunohistochemistry and fluorescence in situ hybridization (FISH). Clinical characteristics, therapeutic options and clinical follow-up data were also analyzed. Back pain was the most common presenting symptom. The main pathological morphology observed was papillae embedded in a myxoid background, but other rare morphologies were also present. Immunostaining revealed epidermal growth factor receptor (EGFR) expression in four MPE, while FISH for EGFR was negative. No correlation between tumor recurrence and EGFR overexpression was found. Ultrastructural examination revealed adherens junctions and intracytoplasmic lumina with microvilli. Patients with gross-total resection (GTR) had no tumor recurrence (p = 0.021). Also, patients with subtotal resection (STR) followed by radiotherapy showed a higher local control rate than patients with STR alone (p = 0.043). The diagnosis of MPE should be made considering the histology, immunohistochemistry, imaging studies and anatomical site. GTR of the tumor or STR followed by radiotherapy are more likely to avoid tumor recurrence than STR alone. Based on our findings, there is no correlation between tumor recurrence and EGFR expression.     

The prognostic implications of Hyam’s subtype for patients with Kadish stage C esthesioneuroblastoma

Esthesioneuroblastoma (EN) is a rare sinonasal tumor with varied aggressiveness and potential for intracranial invasion. EN is staged anatomically with radiographic evaluation using the Kadish staging system (stages A, B, and C) and histologically by using Hyam’s criteria (grades 1–4). Here we show that despite radiographic evidence of aggressive features, the prognosis of patients with Kadish stage C EN is best predicted by tumor histology using Hyam’s criteria. We retrospectively analyzed patients with EN with Kadish stage C who were evaluated and treated at our institution between 1995 and 2009. Clinical information was collected using patient medical records, imaging, and review of pathological specimens. Twenty patients with Kadish stage C EN were identified with mean age of 51 years (31–70 years) with a median follow-up of 41.4 months (1.3–175 months). Upon pathological review, 44.4% of patients had low-grade (1/2) and 55.6% had high-grade (3/4) histology. About 37.5% of patients with low-grade EN had undergone gross total resection (GTR) and the remaining 62.5% had GTR and adjuvant radiation, whereas 50% of patients with high-grade ER had undergone GTR, 20% had undergone GTR and adjuvant radiation, and 30% had been treated with a subtotal resection (STR) and adjuvant radiation. The 5-year and 10-year survival in patients with low-grade EN was 86% in comparison to 56% and 28% with high-grade EN, respectively. In patients with low-grade EN, the 2-year progression free survival (PFS) was 86% and the 5-year PFS was 65% in comparison to 73% and 49% in patients with high-grade EN, respectively. The patient’s tumor histology (Hyam’s criteria) appeared to be the best way of predicting the prognosis and for selecting patients for adjuvant radiotherapy.     

Early or late radiotherapy following gross or subtotal resection for atypical meningiomas: Clinical outcomes and local control

We report a single institution series of surgery followed by either early adjuvant or late radiotherapy for atypical meningiomas (AM). AM patients, by WHO 2007 definition, underwent subtotal resection (STR) or gross total resection (GTR). Sixty-three of a total 115 patients then received fractionated or stereotactic radiation treatment, early adjuvant radiotherapy (≤4 months after surgery) or late radiotherapy (at the time of recurrence). Kaplan Meier method was used for survival analysis with competing risk analysis used to assess local failure. Overall survival (OS) at 1, 2, and 5 years for all patients was 87%, 85%, 66%, respectively. Progression free survival (PFS) at 1, 2, and 5 years for all patients was 65%, 30%, and 18%, respectively. OS at 1, 2, and 5 years was 75%, 72%, 55% for surgery alone, and 97%, 95%, 75% for surgery + radiotherapy (log-rank p-value = 0.0026). PFS at 1, 2, and 5 years for patients undergoing surgery without early adjuvant radiotherapy was 64%, 49%, and 27% versus 81%, 73%, and 59% for surgery + early adjuvant radiotherapy (log-rank p-value = 0.0026). The cumulative incidence of local failure at 1, 2, and 5 years for patients undergoing surgery without early External Beam Radiation Therapy (EBRT) was 18.7%, 35.0%, and 52.9%, respectively, versus 4.2%, 13.3%, and 20.0% for surgery and early EBRT (p-value = 0.02). Adjuvant radiotherapy improves OS in patients with AM. Early adjuvant radiotherapy improves PFS, likely due to the improvement in local control seen with early adjuvant EBRT.     

Long term results of multimodality treatment of craniopharyngioma in children

The management of craniopharyngioma has been controversial for years. We review our 20 year experience in the treatment of paediatric craniopharyngioma. Twenty-five patients were treated for craniopharyngioma at The Montreal Children's Hospital from 1972 to 1991. They included 15 males and 10 females whose median age was 10 years. The initial clinical manifestations were remarkable for a predominance of symptoms and signs related to intracranial hypertension, followed in frequency by visual and endocrinological deficits. Radiologically, there were five cystic craniopharyngiomas, one solid and 19 mixed. Several therapeutic approaches were used including stereotactic drainage followed by radiotherapy or radiosurgery (three cases), transsphenoidal removal (six cases) and subfrontal and/or pterional craniotomy for total (three cases) or partial (13 cases) removal, followed by radiotherapy in 10 cases. The follow up period averaged 11 years. Eight patients recurred, three after total and three after partial removal; none of these six patients had received radiotherapy. Two cases treated by stereotactic drainage recurred, one received adjuvant radiosurgery and the other conventional radiotherapy. Morbidity was lower, and quality of survival better, with more conservative approaches. More conservative approaches in the management of craniopharyngioma are reasonable alternatives for treatment. Similar rates of disease control are observed with less morbidity and better quality of survival.     

MIB-1 labeling index predicts recurrence in intraventricular central neurocytomas

Despite the relatively low-grade of most central neurocytomas (CN), evidence suggests the existence of an aggressive subset with a propensity for recurrence. Recent studies have found the MIB-1 labeling index to be a prognostic indicator in CN. Here we review our experience with CN to analyze the relationships between extent of resection, adjuvant therapy, tumor histology, and clinical outcomes based on aggressive histology, as defined by MIB-1 labeling. A retrospective review was performed on histologically proven CN surgically resected from 1993 to 2009 at the University of California at San Francisco. Recurrence rates were analyzed using the Kaplan–Meier method with respect to MIB-1 labeling and extent of resection. All MIB-1 labeling indices were analyzed. A total of 18 patients were identified with a mean age of 30 years (range 17–58 years) and median follow-up of 40 months (5–173 months). The treatments were: gross total resection (GTR) alone (17% of patients), subtotal resection (STR) alone (50% of patients), STR plus radiotherapy (XRT: external beam or stereotactic radiosurgery: 28% of patients), or STR plus chemotherapy (5% of patients). The extent of resection and a MIB-1 labeling index >4% was predictive of recurrence (p < 0.01). In the 33% of the patients in whom the tumor recurred, all had STR with MIB-1 labeling >4% with median time to recurrence of 23.5 months. The 2-year and 4-year recurrence rates in patients with MIB-1 labeling >4% were 50% and 75% respectively. No patient with a MIB-1 labeling index <4% who received STR alone had a recurrence. Thus, in patients with CN who were treated with STR, histology demonstrating a MIB-1 labeling index >4% can be a clinically useful prognostic indicator and can help guide adjuvant treatment.     

Clinical Outcome of Treatment for Patients with Giant Cell Tumor in Spine

Objective

The treatment of giant cell tumor (GCT) is mainly performed surgically. However, GCT in spine seems difficult to treat because of the limited surgical accessibility and proximity. In this report, we analyzed the outcome of GCT treatment in spine.

Methods

Between 2000 and 2012, 19 patients received treatment for GCT in spine. Median age at their first diagnosis was 31 years, 10 patients were male, and 9 female. Fourteen tumors were located in the sacrum, 1 in cervical, 1 in thoracic and 3 in lumbar spine. As primary treatment, gross total removal (GTR) was done in 6 patients, and subtotal removal (STR) in 13 patients. Radiation therapy (RT) as an adjuvant therapy was performed in 2 cases in GTR group and 10 cases in STR group.

Results

During the follow-up, 7 patients had local recurrence (36.8%). The average period until recurrence after primary treatment was 14 months. No recurrence was detected in GTR group. Recurrence was noted in 7 out of 13 patients who underwent STR. These differences were statistically significant (p=0.024). A median of recurrence free period (RFP) was 84 months. Also average RFP of the RT group was 112 months, and non-RT group was 65 months. These differences were statistically significant (p=0.041).

Conclusion

Treatment of choice for GCT in spine is a complete removal of tumor without neurological deficits. In case of incomplete removal, radiation therapy may be a useful adjuvant treatment modality.     

Glioneuronal tumors of cerebral hemisphere in children: correlation of surgical resection with seizure outcomes and tumor recurrences

Object

Glioneuronal tumors are common neoplasms among the cerebral hemisphere during childhood. They consist of several histological types, of which gangliogliomas (GGs) and dysembryoplastic neuroepithelial tumors (DNTs) are most common and often present with seizures. A great majority of glioneuronal tumors are benign. However, there are conflict reports regarding postoperative tumor recurrence rates and seizure control. The authors analyzed and compared these tumors for their locations and histology and the tumor and seizure control following resection.

Methods

The authors conducted a retrospective analysis of patients with pediatric glioneuronal tumors in the cerebral hemisphere. All histology reports and neuroimaging are reviewed. Seizure group and non-seizure group were compared with their tumor types and locations. The extent of tumor resections were divided into gross total resection (GTR) and subtotal resection (STR). Postoperative tumor recurrence-free survival (RFS) and seizure-free survival for patients who had the initial surgery done at our institution were calculated using Kaplan-Meier method.

Results

There were 90 glioneuronal tumors including 58 GGs, 22 DNTs, 3 papillary glioneuronal tumor, 3 desmoplastic infantile gangliogliomas, 3 anaplastic GGs, and 1 central neurocytoma. Seventy-one patients (seizure group) presented with seizures. The temporal lobe is the most common location, 50 % in this series. GTR was attained in 79 patients and STR in 11. All of the patients with GTR had lesionectomy, and only six of them had extended corticectomy or partial lobectomy. Postoperative seizure outcome showed that 64 (90 %) of seizure group had Engel’s class I, but five patients subsequently developed recurrent seizures. Patients with DNTs had a higher seizure recurrence rate. Tumor RFS was 87 % at 5 years and 75.5 % at 10 years. There are no significant difference in tumor recurrences between GGs and DNTs (p?=?0.876). Comparison between GRT (67) and STR (9) showed that in spite of the better 5-year tumor RFSs among GRT group (94 %) than STR group (66 %), the 10-year RFSs showed no significant difference between GRT and STR groups (p?=?0.719). Recurrent seizures are often related to recurrent tumor.

Conclusion

Lesionectomy alone often provides a high-rate seizure freedom. GGs and DNTs are benign tumor, but recurrences of GGs and DNTs are not uncommon. They may show late recurrences in spite of GTR. These patients need longer follow-up for 10 years. Recurrent seizures are often related to a tumor recurrence.

     

Review of controversies in management of non-benign meningioma

Meningiomas are one of the most common brain tumors. World Health Organisation (WHO) Grade II and Grade III meningiomas are grouped together as non-benign meningioma (NBM). There are several controversies surrounding NBM management, including the significance of extent of resection and the efficacy of post-operative radiation and drug treatment. We reviewed the literature to develop recommendations for management of NBM. The questions we sought to answer were: Does gross total resection (GTR) improve patient outcome? Is radiation therapy (RT) warranted after complete or after incomplete resection of NBM? What drug therapies have been proven to improve outcome in patients with NBM? We found that GTR improves outcome in WHO Grade II meningioma, and should be attempted whenever considered safe. GTR correlates less closely to outcome in Grade III meningioma compared to subtotal resection (STR). Extreme measures to completely resect Grade III meningioma are not warranted. RT following GTR of Grade II meningioma does not improve patient outcome, and may be reserved for recurrence. RT improves outcome following STR of Grade II meningioma. RT improves outcome after resection of Grade III meningioma. No drug therapy has been shown to improve outcome in NBM. This review elucidates recommendations for some of the controversies involving NBM.     

Efficacy of temozolomide as adjuvant chemotherapy after postsurgical radiotherapy alone for glioblastomas

Objectives

The aim of this study was to assess the efficacy of adjuvant TMZ chemotherapy for newly diagnosed GBM patients who were treated with surgery followed by radiotherapy alone.

Material and methods

Between January 2003 and April 2005, 59 consecutive GBM patients underwent radiation therapy after surgical resection and subsequently received TMZ chemotherapy. For the comparative analysis, we selected 60 clinically matched GBM patients who underwent radiotherapy followed by nitrosourea-based chemotherapy (NUBC), at the same institution between June 1995 and April 2005. The study cohort was divided into two groups, those with adjuvant TMZ treatment and with NUBC.

Results

59 patients with adjuvant TMZ treatment were assigned to the treatment group and 60 patients with NUBC to the control group. The median overall survival for the treatment group was 18.2 months (95% CI, 11.7–24.7 months), compared with the survival of 14.5 months (95% CI, 11.2–17.7 months) for the control group (p = 0.019). The progression-free survival for the treatment group was 5.6 months (95% CI, 4.4–6.7 months), while the control group showed progression-free survival of 3.3 months (95% CT, 3.2–6.0 months) (p = 0.030). Uni- and multivariate analysis revealed that extent of surgical resection, age ≥55 years and postoperative KPS were significantly associated with survival.

Conclusion

Adjuvant TMZ chemotherapy provided a clinically relevant benefit of survival, as compared with NUBC. Thus, we suggest that adjuvant TMZ chemotherapy may be effective even for patients who did not receive concomitant chemoradiotherapy for GBM.     

Craniopharyngioma in children: Marseille experience

Objectives The management of craniopharyngioma in children represents a challenging problem. If radical excision is recommended by many authors as the initial treatment, in some cases, particularly in recurrent tumours, other methods (gamma knife surgery and intracystic bleomycin) can be very useful. Even if craniopharyngioma is a benign tumour, recurrences are frequent, and the aim of our study was to analyse our results, to try to determine some prognostic factors of recurrences and to discuss about a new strategy concerning the initial management of these tumours.Methods Forty-seven children with craniopharyngioma were treated in the Department of Pediatric Neurosurgery. All of the patients, but five children treated by intracystic bleomycin, underwent a surgical resection of the tumour as initial treatment with the goal of achieving gross total removal (GTR) of the tumour. Two children had radiotherapy and gamma knife treatment, respectively, following surgery for a tumoural residue. All the children had a magnetic resonance imaging (MRI) study 3 months after surgery to evaluate the results of the initial treatment. Using statistical analysis, some prognostic factors (age, sex, location, aspect, size of the tumour and result of the first MRI) have been studied.Results Forty-two children were operated on, but one died in the immediate postoperative period from a major stroke due to carotid spasm. GTR, defined as the absence of residue on the first MRI control, was achieved in 27 children (65.8%), but 7 patients (25.9%) presented recurrence. Subtotal removal (STR) was obtained in 14 children (34.2%), but 9 patients (64.3%) developed a recurrence defined as the growth of the residual tumour with or without clinical symptoms. Five children having a small- or moderate-size cystic craniopharyngioma were treated using one-stage (three cases) or two-stage (two cases) intracystic bleomycin and any presented recurrence. All the prognostic factors studied, except one (presence of a residue on the first MRI control), do not have a statistical significance.Conclusion Craniopharyngioma in children remains a formidable tumour, and regardless of whatever progress made in their management, the incidence of recurrences is still elevated and severe sequelae can be observed. There are no prognostic factors among those studied concerning the recurrences of these tumours except the quality of the exeresis confirmed by the first postoperative MRI.     

Clinical features and long-term outcomes of intraspinal ependymomas in pediatric patients

Purpose

The aim of this study was to discuss the clinical manifestations, radiological features, treatment, and long-term outcomes of intraspinal ependymomas (Word Health Organization grade II) in pediatric patients.

Methods

The data of 15 pediatric patients who underwent microsurgery for intraspinal grade II ependymomas were retrospectively reviewed. Pre- and postoperative magnetic resonance imaging was performed in all patients. The diagnosis of grade II ependymomas was based on pathology. All the follow-up data were obtained during office visits.

Results

There were ten males and five females, with a mean age of 13.7?±?3.4 years. Four tumors were located in the cervical cord, six in the cervicothoracic cord, four in the thoracic cord, and one in the conus-cauda region, respectively. The most common symptom was motor deficits. Gross total resection (GTR) of the tumor was achieved in 12 cases, and subtotal resection (STR) was achieved in three cases. Regrowth of the residual tumor was observed in two STR cases during a mean follow-up period of 44.8 months. STR was performed again in one case due to clinical progression. At the last follow-up, 12 patients experienced an improvement in the neurological function and three patients maintained their preoperative status.

Conclusions

Pediatric intraspinal grade II ependymomas are amenable to surgical resection before neurological deficits deteriorate. GTR is the best treatment of choice, and the outcome is favorable. Due to uncertain therapeutic efficacy and possible radiation-induced toxicity, postoperative radiotherapy should be considered carefully for cases of STR.     

Clinical features and post-surgical outcome of patients with astroblastoma

Astroblastoma is a rare tumor, and thus experience with these lesions is very limited. The prognosis and appropriate treatment is not well understood, as few individual centers have enough experience with astroblastoma to guide treatment recommendations. We performed a systematic comprehensive search of the published English language literature on patients undergoing surgery for astroblastoma to summarize what is known about these tumors, and to provide some framework for future efforts in this area. A total of 62 references met our inclusion criteria, and contained individual patient data on 116 patients with astroblastoma. Determination of overall survival rates was performed using Kaplan-Meier analysis. This analysis suggests that the distribution is bimodal, with a prominent peak in young adulthood. Astroblastomas are generally amenable to complete tumor resection, even when very large, with gross total resection (GTR) achieved in 71/85 (84%) of reported patients, including both 9 cm tumors reported. Patients undergoing GTR experienced a significant improvement in survival compared to patients who underwent subtotal resection (STR) (5-year progression-free survival: GTR 83% versus [vs.] STR 55%, log rank p = 0.011). Although patients receiving external beam radiotherapy or fractionated three-dimensional conformal radiotherapy (XRT) seemed to have lower survival rates, this was not statistically significant (5-year survival: GTR 94% vs. GTR + XRT 73%, log rank p = 0.463). Thus, we have reported the results of a summary of the literature on astroblastomas and have accurately described outcome characteristics using a data set that would be difficult to accumulate at a single center treating this tumor.     

A Long-Term Survival Case of a Primary Malignant Intracerebral Nerve Sheath Tumor

We report a long-term survival case of a primary malignant intracerebral nerve sheath tumor (MINST) occurring in the right frontal lobe of a 13-year old boy. After the gross total resection (GTR), we have performed radiation therapy but it recurred 50 months after the surgery, so the second GTR was performed. Later, second tumor recurrence was found 4 months after the second surgery. Subsequently the third GTR, radiotherapy, and chemotherapy were carried out. At present, the patient has been remaining alive for 77 months without evidence of tumor recurrence. According to the previous reports, the primary MINST is very rare : there are only 8 cases reported. It is also a fast-growing, invasive tumor with poor outcome. This is the first case that had no recurrence for 50 months after the surgery among the reported cases that had been followed up for more than 5 years. It is supposed that a period of recurrence free survival after GTR and low mitotic activity are associated with the patient''s prognosis. A GTR followed by adjuvant radiation therapy and chemotherapy will be recommended to patients of MINST.     

手术联合125I粒子植入治疗复发性脑胶质瘤及预后因素初步分析

目的探讨手术联合125I粒子植入治疗复发性脑胶质瘤的疗效及影响预后的主要因素。方法 60例手术加放化疗后复发的脑胶质瘤患者随机分为手术联合125I粒子植入组35例,单纯手术对照组25例进行治疗,术后每2个月复查MRI进行随访,收集临床资料和随访结果进行分析。用Kaplan-Meier法计算全组的累积生存率,Log-Rank检验做单因素分析,Cox比例风险回归模型进行多因素分析。结果全组随访32~128周,平均随访时间52.8周,125I粒子植入组中位生存期60.3周(95%CI,53.3~67.3周),1 a生存率为71.4%,单纯手术组中位生存期43.1周(95%CI,37.5~48.8周),1 a生存率为36%(P<0.05)1。25I粒子植入组中WHO病理分级Ⅱ、Ⅲ、Ⅳ期的患者1 a生存率分别为87.8%、65.3%、36.4%(P<0.01);肿瘤全切组和次全切的患者1 a生存率分别为79.2%和54.5%(P<0.05)。单因素分析显示,肿瘤组织学分级、肿瘤部位、肿瘤切除程度和放射性并发症是手术联合125I粒子植入治疗复发性胶质瘤的预后影响因素;多因素分析显示肿瘤组织学分级和肿瘤切除程度是影响预后的独立因素。结论手术联合125I粒子植入可有效地延缓脑胶质瘤的生存时间,提高患者的生存率,肿瘤组织学分级和肿瘤切除程度是影响其预后的最重要因素。     

多学科协作模式在肢端肥大症中的应用

目的比较多学科协作模式(multi-disciplinary team,MDT)与传统单一外科模式诊治肢端肥大症的疗效差异,探讨MDT模式对于肢端肥大症的诊疗价值。方法回顾性分析245例肢端肥大症患者,根据诊治模式分为传统单一学科模式组(对照组)和MDT组,比较两组的随访率、合并症检出率、手术入路选择、手术全切率、内分泌缓解率、并发症发生率等的差异。结果 MDT组的合并症检出率高于对照组;MDT组的经颅手术比例有较对照组下降的趋势(1.4%vs 6.7%,χ~2=3.39,P=0.066);MDT组的全切率高于对照组(80.3%vs.65.1%,χ~2=6.184,P=0.013);两组的手术后内分泌缓解率无显著差异(70.1%vs.59.3%,χ~2=2.644,P=0.104);综合治疗后MDT组的末次随访内分泌缓解率高于对照组(81.1%vs.67.4%,χ~2=5.185,P=0.023)。结论 MDT模式能够提高肢端肥大症患者的合并症检出率,降低经颅手术比例,提高手术全切率及综合治疗后内分泌缓解率,优于传统的单一学科诊疗模式。