Category: Oncology

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Hemangiopericytomas grade II are not benign tumors

Acta Neurochir (2011) 153:385–394.DOI 10.1007/s00701-010-0877-1

Hemangiopericytomas (HPs) of the central nervous system are rare tumors and afflicted with a high propensity of recurrences and metastases. Histopathologically, HPs correspond to differentiated (WHO grade II) and anaplastic (WHO grade III) tumors. With respect to the available literature and our own experiences, the aggressiveness, especially of differentiated grade II HPs, seems to be underestimated. Methods Thus, in this retrospective study, we describe tumor behavior and examined the effect of radio- and chemotherapy on tumor control with respect to the WHO classification of grade II and III neoplasms. This study consists of 15 patients with cerebral (n=10) and spinal (n=5) HPs. Results Seven HPs were histopathologically classified as grade II and eight as anaplastic grade III tumors. Complete surgical resection could be achieved in 60% of cerebral and in 25% of spinal HPs. In total, local recurrences occurred in 20% of patients within 17.3 months after the primary operation. Recurrences occurred both from differentiated (n=1) and anaplastic (n=2) neoplasms. Treatment comprised re-operation followed by radio- and chemotherapy. Pointing out the importance of the extent of surgical resection, in this study, we could not detect a single patient showing any recurrences or systemic metastases after complete surgical resection of grade II HPs. During primary diagnostics, four patients showed systemic metastases. Although these tumors could be controlled via surgery, systemic metastases appeared in further four patients within 60.4 months. Interestingly, two of them were classified as differentiated tumors (WHO grade II). To control tumor progress, radiotherapy seemed to be partially effective. On the other hand, however, chemotherapy did not show any effect on tumor control. With respect to these results, screening investigations seem to be indispensable and are highly recommended during primary diagnostics and after the appearance of recurrences or metastases, independent of the histopathological staging of the tumor. Conclusion With respect to our results, radical surgical resection offers the best treatment option to control tumor progress. In case of subtotal resection or histopathologically diagnosed anaplasia (WHO III), radiotherapy seems to be indicated; however, chemotherapy did not show effectiveness to control tumor progress.

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Molecular biology of familial and sporadic vestibular schwannomas: implications for novel therapeutics

J Neurosurg 114:359–366, 2011.DOI: 10.3171/2009.10.JNS091135

Vestibular schwannomas (VSs) are benign tumors arising from the sheath of cranial nerve VIII. The pathogenesis underlying most familial and sporadic VSs has been linked to a mutation in a single gene, the neurofibromin 2 (NF2) gene located on chromosome 22, band q11–13.1.

In this review, the authors summarized what is known about the epidemiology of NF2 mutations and patients with VSs. The authors also discuss the function of the NF2 gene product, merlin, and describe the known and hypothetical effects of genetic mutations that lead to merlin dysfunction on a broad variety of cellular and histological end points.

A better understanding of the molecular pathobiology of VSs may lead to novel therapeutics to augment current modalities of treatment while minimizing morbidity.

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Surgical approaches to hypothalamic hamartomas

Neurosurg Focus 30 (2):E2, 2011.(DOI: 10.3171/2010.11.FOCUS10250)

Hypothalamic hamartomas (HHs) are devastating lesions causing refractory epilepsy, rage attacks, social ineptitude, and precocious puberty. Microsurgical and/or endoscopic resection offers an excellent risk/benefit profile for cure or improvement of epilepsy.

Methods. The authors reviewed a prospective database maintained during the first 7 years of the Barrow Hypothalamic Hamartoma program. They describe and illustrate their surgical methods, and they review data from several previous publications regarding surgical outcome.

Results. To date, the authors have performed surgery in 165 patients for symptomatic HHs. Patients underwent an endoscopic, transcallosal, or skull base approach, or multiple approaches. Twenty-six patients (15.8%) required more than 1 treatment for their HH.

Conclusions. Microsurgical and endoscopic resection of symptomatic HHs are technically demanding but can be performed safely with excellent results and an acceptable risk profile. Meticulous attention to the subtleties of surgical management helps optimize outcomes.

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Endocrinologic, neurologic, and visual morbidity after treatment for craniopharyngioma

J Neurooncol (2011) 101:463–476. DOI 10.1007/s11060-010-0265-y

Craniopharyngiomas are locally aggressive tumors which typically are focused in the sellar and suprasellar region near a number of critical neural and vascular structures mediating endocrinologic, behavioral, and visual functions. The present study aims to summarize and compare the published literature regarding morbidity resulting from treatment of craniopharyngioma.

We performed a comprehensive search of the published English language literature to identify studies publishing outcome data of patients undergoing surgery for craniopharyngioma. Comparisons of the rates of endocrine, vascular, neurological, and visual complications were performed using Pearson’s chi-squared test, and covariates of interest were fitted into a multivariate logistic regression model.

In our data set, 540 patients underwent surgical resection of their tumor. 138 patients received biopsy alone followed by some form of radiotherapy. Mean overall follow-up for all patients in these studies was 54 ± 1.8 months. The overall rate of new endocrinopathy for all patients undergoing surgical resection of their mass was 37% (95% CI = 33– 41). Patients receiving GTR had over 2.5 times the rate of developing at least one endocrinopathy compared to patients receiving STR alone or STR + XRT (52 vs. 19 vs. 20%, v2 P<0.00001). On multivariate analysis, GTR conferred a significant increase in the risk of endocrinopathy compared to STR + XRT (OR = 3.45, 95% CI = 2.05–5.81, P<0.00001), after controlling for study size and the presence of significant hypothalamic involvement. There was a statistical trend towards worse visual outcomes in patients receiving XRT after STR compared to GTR or STR alone (GTR = 3.5% vs. STR 2.1% vs. STR + XRT 6.4%, P = 0.11). Given the difficulty in obtaining class 1 data regarding the treatment of this tumor, this study can serve as an estimate of expected outcomes for these patients, and guide decision making until these data are available.

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Categorization and characterization of lesions of the orbital apex

Neuroradiology (2011) 53:89–107. DOI 10.1007/s00234-010-0712-7

The apex of the orbit is formed by the union of the lesser and greater wings of the sphenoid bone and acts as an osseous tunnel for numerous neurovascular structures entering the orbit from the cranial vault. Lesions of the orbital apex are clinically important as they can have an adverse effect on vision. A broad range of lesions can occur here, and our purpose is to organize the pathologic processes which occur in the orbital apex into logical imaging differentials, establish an organized approach to image analysis, and present examples of representative lesions.

Methods We review the anatomy of the orbital apex and categorize and describe the pathologic entities that are encountered most frequently in this anatomically compact region and identify imaging patterns that can help to narrow the differential diagnosis.

Results Categories of orbital apex lesions include neoplasms, inflammatory processes, infections, lesions causing extrinsic compression, and vascular lesions. This categorization provides an organized framework to facilitate a reasonable differential diagnosis. Computed tomography and magnetic resonance imaging are the modalities of choice to evaluate and characterize orbital apex lesions, and imaging examples utilizing these modalities will be presented.

Conclusion The orbital apex is a clinically important anatomical region and hosts diverse pathologic processes. An awareness of common imaging patterns can help to generate a focused differential diagnosis. A systematic categorical approach can be of help to radiologists attempting to accurately characterize lesions in this area.

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Decision analysis of treatment options for vestibular schwannoma

J Neurosurg 114:400–413, 2011. (DOI: 10.3171/2010.3.JNS091802)

Widespread use of MR imaging has contributed to the more frequent diagnosis of vestibular schwannomas (VSs). These tumors represent 10% of primary adult intracranial neoplasms, and if they are symptomatic, they usually present with hearing loss and tinnitus. Currently, there are 3 treatment options for quality of life (QOL): wait and scan, microsurgery, and radiosurgery. In this paper, the authors’ purpose is to determine which treatment modality yields the highest QOL at 5- and 10-year follow-up, considering the likelihood of recurrence and various complications.

Methods. The MEDLINE, Embase, and Cochrane online databases were searched for English-language articles published between 1990 and June 2008, containing key words relating to VS. Data were pooled to calculate the prevalence of treatment complications, tumor recurrence, and QOL with various complications. For parameters in which incidence varied with time of follow-up, the authors used meta-regression to determine the mean prevalence rates at a specified length of follow-up. A decision-analytical model was constructed to compare 5- and 10-year outcomes for a patient with a unilateral tumor and partially intact hearing. The 3 treatment options, wait and scan, microsurgery, and radiosurgery, were compared.

Results. After screening more than 2500 abstracts, the authors ultimately included 113 articles in this analysis. Recurrence, complication rates, and onset of complication varied with the treatment chosen. The relative QOL at the 5-year follow-up was 0.898 of normal for wait and scan, 0.953 for microsurgery, and 0.97 for radiosurgery. These differences are significant (p < 0.0052). Data were too scarce at the 10-year follow-up to calculate significant differences between the microsurgery and radiosurgery strategies.

Conclusions. At 5 years, patients treated with radiosurgery have an overall better QOL than those treated with either microsurgery or those investigated further with serial imaging. The authors found that the complications associated with wait-and-scan and microsurgery treatment strategies negatively impacted patient lives more than the complications from radiosurgery. One limitation of this study is that the 10-year follow-up data were too limited to analyze, and more studies are needed to determine if the authors’ results are still consistent at 10 years.

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Development of Stereotactic Mass Spectrometry for Brain Tumor Surgery

Neurosurgery 68:280–290, 2011 DOI: 10.1227/NEU.0b013e3181ff9cbb

Surgery remains the first and most important treatment modality for the majority of solid tumors. Across a range of brain tumor types and grades, postoperative residual tumor has a great impact on prognosis. The principal challenge and objective of neurosurgical intervention is therefore to maximize tumor resection while minimizing the potential for neurological deficit by preserving critical tissue.

OBJECTIVE: To introduce the integration of desorption electrospray ionization mass spectrometry into surgery for in vivo molecular tissue characterization and intraoperative definition of tumor boundaries without systemic injection of contrast agents.

METHODS: Using a frameless stereotactic sampling approach and by integrating a 3-dimensional navigation system with an ultrasonic surgical probe, we obtained image-registered surgical specimens. The samples were analyzed with ambient desorption/ ionization mass spectrometry and validated against standard histopathology. This new approach will enable neurosurgeons to detect tumor infiltration of the normal brain intraoperatively with mass spectrometry and to obtain spatially resolved molecular tissue characterization without any exogenous agent and with high sensitivity and specificity.

RESULTS: Proof of concept is presented in using mass spectrometry intraoperatively for real-time measurement of molecular structure and using that tissue characterization method to detect tumor boundaries. Multiple sampling sites within the tumor mass were defined for a patient with a recurrent left frontal oligodendroglioma, World Health Organization grade II with chromosome 1p/19q codeletion, and mass spectrometry data indicated a correlation between lipid constitution and tumor cell prevalence.

CONCLUSION: The mass spectrometry measurements reflect a complex molecular structure and are integrated with frameless stereotaxy and imaging, providing 3-dimensional molecular imaging without systemic injection of any agents, which can be implemented for surgical margins delineation of any organ and with a rapidity that allows real-time analysis.

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Long-term Results of the Neuroendoscopic Management of Colloid Cysts of the Third Ventricle: A Series of 90 Cases

Neurosurgery 68:179–187, 2011 DOI: 10.1227/NEU.0b013e3181ffae71

The endoscopic removal of third ventricular colloid cysts has been developed as an alternative to microsurgical transcortical-transventricular and transcallosal approaches.

OBJECTIVE: To examine the value of endoscopic technique by reviewing the large number of endoscopically treated patients with long-term follow-up in 2 neurosurgical centers.

METHODS: A retrospective chart review was conducted for all patients admitted for resection of a third ventricular colloid cyst to the Radboud University Nijmegen Medical Centre (Nijmegen, the Netherlands) and the Hoˆ pital Henri Mondor (Paris, France) between 1994 and 2007. Both clinical and radiological symptoms and operative results were evaluated.

RESULTS: Postdischarge clinical follow-up was available for 85 patients over a mean period of 4 years 3 months. Permanent morbidity occurred in 1 patient (persisting preoperative memory deficit). Follow-up imaging of 80 evaluable patients showed that total or nearly total cyst removal was possible in 46 individuals (57.5%). Residual cyst was present in 34 patients (42.5%), and 6 required repeated endoscopic surgery for symptomatic regrowth. Recurrent cysts were mainly seen within the first 2 years after surgery.

CONCLUSION: It is debatable whether the higher numbers of recurrent or residual cysts can be justified by the slightly lower complication rates achieved with endoscopic removal. However, results have been improving over the years. Moreover, the modifications observed on control magnetic resonance images justify the need for regular control imaging for at least the first 2 years postoperatively.

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Posterior fossa ependymomas: new radiological classification with surgical correlation

Childs Nerv Syst (2010) 26:1765–1772. DOI 10.1007/s00381-010-1251-6

The key determinant of long-term outcome in infratentorial ependymomas remains the extent of surgical resection. We describe a new radiological classification system which is validated against surgical findings and correlated with risk of post-operative residual tumour.

Methods Twenty-five consecutive patients (12 females, mean age 4.9 years, range 0.5–17 years) with infratentorial ependymomas were studied. Lesions were classified on preoperative MRI according to the pattern of extension, brainstem displacement and involvement of the obex, as lateral-type or midfloor-type tumours. Twenty-one operative records were reviewed with respect to the microanatomical tumour origin by a paediatric neurosurgeon, blinded to MRI findings. Follow-up imaging studies were evaluated for residual tumour.

Results There were 15 cases of midfloor-type tumour (anterior displacement of brainstem, infiltration of obex) and 10 cases of lateral-type tumour (lateral displacement of brainstem, obex free of tumour). Extension into prepontine or cerebellopontine cisterns was more common in lateraltype tumours. Agreement between the radiological classification and tumour origin, as defined by operative records, was seen in 18 out of 20 cases. Risk of residual tumour in lateral-type tumours was more than twice that of midfloortype tumours (80% vs. 33%, p=0.04). Risk of tumour residual was also significantly higher when vessel encasement or prepontine extension was observed.

Conclusions Infratentorial ependymomas can be preoperatively classified as lateral-type or midfloor-type tumours. This correlates well with operative findings. Lateral-type tumours have significantly increased risk of residual tumour compared to midfloor- type tumours and this may influence intensity of imaging surveillance.

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Long-term Outcome After Resection of Intraspinal Ependymomas: Report of 86 Consecutive Cases

Neurosurgery 67:1622–1631, 2010 DOI: 10.1227/NEU.0b013e3181f96d41

Objective: To evaluate progression-free survival, overall survival (OS) and long-term clinical outcome in a consecutive series of 86 patients with intraspinal ependymomas.

METHODS: Medical charts were retrospectively reviewed. Surviving patients voluntarily participated in a clinical history and physical examination that focused on neurological function and current tumor status.

RESULTS: Follow-up data are nearly 100% complete; mean follow-up time was 82 months. Eighty-five patients (99%) had surgery as a first-line treatment; 14 (17%) of these patients received adjuvant radiotherapy. Of the 85 patients who underwent primary surgery, gross total resection was performed in 60 patients (71%) and subtotal resection in 25 patients (29%). Ten-year progression-free survival rate was 75%; 5-year OS, 97%; and 10-year OS, 91%. Reduced preoperative neurological function and older age at diagnosis were significantly associated with increased risk of death. At follow-up, spontaneous regression of residual tumor after primary surgery may have occurred in 7 of 19 patients (37%). More than 75% of patients had neurological function compatible with an independent life at follow-up. Good preoperative neurological function was significantly associated with favorable outcome. It was not possible to evaluate the effect of radiotherapy on progression-free survival and OS.

CONCLUSION: Gross total resection remains the optimal treatment for patients with spinal ependymoma. Patients should be monitored with a clinical examination and magnetic resonance imaging at regular intervals up to 10 years after surgery.

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Vascular Endothelial Growth Factor: The Major Factor for Tumor Neovascularization and Edema Formation in Meningioma Patients

Neurosurgery 67:1703–1708, 2010 DOI: 10.1227/NEU.0b013e3181fb801b

Peritumoral brain edema (PTBE) may be crucial in the clinical outcome of meningioma patients. The underlying pathogenetic key mechanism has so far not been determined. Sex, age, tumor size, location, involvement of other structures, or the histological appearance was not found to sufficiently explain PTBE formation in meningiomas.

OBJECTIVE: As PTBE formation is widely accepted to be vasogenic, we investigated the role of vascular endothelial growth factor (VEGF) and pial supplying vessels in a series of World Health Organization (WHO) grade I meningiomas.

METHODS: A total of 79 patients with WHO grade I meningiomas were immunohistochemically studied for VEGF and MIB-1. Pre- and postoperative magnetic resonance imaging including 3-dimensional reconstruction of 1.3-mm thick layers, with calculation of tumor and edema volume, was performed. Intraoperatively, the vascular supply and arachnoidal state were noted by the neurosurgeon.

RESULTS: VEGF was found to be exclusively confined to meningioma tumor cells. We identified 4 different patterns. VEGF and supplying pial vessels were found in 14 meningioma patients, pial vascular supply only in 3, VEGF expression only in 46, and neither VEGF expression nor supplying pial vessels in 16. Only the occurrence of both pial vascular supply and tumor VEGF expression was found to be correlated with PTBE formation (P , .002).

CONCLUSION: Our data suggest that VEGF may be crucial in angiogenesis and therefore indirectly in PTBE formation in World Health Organization grade I meningiomas

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A Critical Evaluation of Vestibular Schwannoma Surgery for Patients Younger Than 40 Years of Age

Neurosurgery 67:1646–1654, 2010 DOI: 10.1227/NEU.0b013e3181f8d3d3

There are few published prospective data sets specifically focusing on patients younger than 40 years old undergoing microsurgery for vestibular schwannoma.

OBJECTIVE: We describe functional outcomes and long-term tumor control after surgery in patients younger than 40 years old enrolled in a prospectively collected database over a 25-year period.

METHODS: We selected all vestibular schwannoma patients from a prospectively collected database who were younger than 40 years old at the time of surgical resection for a vestibular schwannoma. Rates of tumor control and hearing preservation were analyzed using Kaplan-Meier analysis, and risk factors for facial nerve palsy, hearing loss, and trigeminal neuropathy were analyzed using multivariate logistic regression.

RESULTS: A total of 204 patients younger than 40 years of age met our inclusion criteria and were included in the analysis. Our data indicate that surgical resection leads to durable long-term freedom from tumor recurrence or progression in 89% of young patients at 15 years of follow-up. Consistent with other published series, hearing was preserved in 68% of patients with smaller tumors (,3 cm). Facial nerve function was preserved in 76% of patients with smaller tumors and 52% of patients with larger tumors (P , .001). On multivariate logistic regression, tumor size was a significant predictor of hearing loss, whereas gross total resection was nearly a significant predictor of hearing loss controlling for other variables (P = .06).

CONCLUSION: We present the largest prospectively studied cohort of young patients undergoing microsurgical resection of vestibular schwannoma. These data suggest that surgical resection provides excellent long-term tumor control in these patients

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Prediction of neurological deficits and recovery after surgery in the supplementary motor area: a prospective study in 26 patients

J Neurosurg 113:1152–1163, 2010.(DOI: 10.3171/2010.6.JNS1090)

Resection of lesions involving the supplementary motor area (SMA) may result in immediate postoperative motor and speech deficits that are reversible in most cases. In the present study the authors aimed to determine the critical involvement of SMA in the lesioned and healthy hemispheres in this functional recovery. They hypothesized that compensatory mechanisms take place following surgery in the SMA, and that these mechanisms can involve either the lesioned or the non-lesioned hemisphere. In addition, they hypothesized that a correlation will be present between the functional MR imaging (fMR) imaging–related activation in the SMA and the occurrence of a functional deficit during intraoperative cortical stimulation.

Methods. Twenty-six patients scheduled for resection of space-occupying lesions involving, or in the vicinity of, the SMA were recruited. Patients underwent an fMR imaging examination that included finger-tapping and verbgeneration tests to assess for motor and language functions. Intraoperatively direct cortical stimulation (DCS) of the SMA region was performed while patients were monitored for language and motor functions using tests similar to those used for the fMR imaging. Task dysfunction during DCS assessed the critical involvement of the SMA in the tested functions. Neurological evaluations were performed prior to surgery and at 3 time points within a month following surgery. A region of interest–based approach was used to evaluate fMR imaging blood oxygen level–dependent activation level and asymmetry in the SMA. These measurements were later compared with the intraoperative DCS and neurological findings.

Results. Functional MR imaging showed greater activation and dominance of the SMA in the lesioned hemisphere in patients who exhibited no motor or language dysfunction during DCS. In addition, patients with the highest activation of the SMA in the lesioned hemisphere for language and motor tests showed stronger coupling of this region with ipsilateral motor and language networks. In contrast, activation in the nonlesioned hemisphere did not correspond with DCS results.

Conclusions. The authors’ findings demonstrate the necessity of activation in the vicinity of the lesioned SMA for functional compensation in motor and language tasks. It is possible that more effective functional coupling of the SMA with motor and language areas in the same hemisphere prevents dysfunctions following surgical intervention. Importantly, fMR imaging activation in the unaffected SMA was not sufficient for development of functional compensation and, if anything, indicated decompensation.

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Morbidity of Transcallosal and Transcortical Approaches to Lesions in and Around the Lateral and Third Ventricles: A Single-Institution Experience

Neurosurgery 67:1483–1496, 2010 DOI: 10.1227/NEU.0b013e3181f7eb68

Resection of an intraventricular mass can result in life-altering complications. Many advocate transcallosal rather than transcortical approaches to these lesions, citing differential postoperative seizure risk.

OBJECTIVE: To test the hypothesis that the complication rates and patient outcomes are no different between these ventricular approaches.

METHODS: The medical records of 127 patients (93 adults and 34 children) operated on for intraventricular lesions between 1996 and 2007 were retrospectively analyzed. Risk factors for specific postoperative complications and outcome were assessed by multivariate analysis.

RESULTS: The transcallosal (59%) or transcortical (41%) approach was used. Gross or nearly total resection was achieved in 87% of cases. The permanent neurological complication rate determined by a staff neurologist was 23.6%. Seizure attributable to surgery occurred after 8% of transcortical and 25% of transcallosal operations (P = .01). After controlling for a variety of factors, the transcallosal approach carried a 4.4-fold increased risk of seizure (95% confidence interval, 1.3-18.9). The operative approach was not a risk factor for any other postoperative complication. One year after surgery, 72% of patients had excellent functional outcome (Karnofsky Performance Score $ 70 and Glasgow Outcome Score = 5). High tumor grade and impaired preoperative Karnofsky Performance Score predicted poor outcome. More than 90% of patients operated on for symptomatic colloid cysts (n = 34) had an excellent outcome.

CONCLUSION: Although the 2 traditional approaches to the ventricular system had similar major complication rates, the transcallosal approach was associated with significantly increased seizure risk. Accordingly, the chosen operative corridor should optimize tumor access and the protection of vulnerable neurovascular structures.

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Treatment decision making based on the published natural history and growth rate of small meningiomas

J Neurosurg 113:1036–1042, 2010, DOI: 10.3171/2010.3.JNS091966

Definitive data allowing clinicians to predict which meningioma patients will fail to respond to conservative management are lacking. To address this need, the authors systematically reviewed the published literature regarding the natural history of small, untreated meningiomas.

Methods. The authors performed a systematic review of the existing literature on untreated meningiomas that were followed with serial MR imaging. They summarize the published linear rates of tumor growth, and the risk factors for development of new or worsened symptoms during follow-up by using a stratified chi-square test.

Results. The search methods identified 22 published studies reporting on 675 patients with untreated meningiomas followed by serial MR imaging. Linear growth rates varied significantly: no growth was the most common rate, although reports of more aggressive tumors noted growth rates of up to a 93% linear increase in size per year. The authors found that few patients with initial tumor diameters < 2 cm went on to develop new or worsened symptoms over a median follow-up period of 4.6 years. Patients with initial tumor diameters of 2–2.5 cm demonstrated a marked difference in the rate of symptom progression if their tumors grew > 10% per year, compared with those tumors growing ≤ 10% per year (42% vs 0%; p < 0.001, chi-square test). Patients with tumors between > 2.5 and 3 cm in initial size went on to develop new or worsened symptoms 17% of the time.

Conclusions. This systematic review of the literature regarding the clinical behavior of untreated meningiomas suggests that most meningiomas ≤ 2.5 cm in diameter do not proceed to cause symptoms in the approximately 5-year period following their discovery. Those that do cause symptoms can usually be predicted with close radiographic follow-up. Based on these findings, the authors suggest the importance of observation in the early course of treatment for small asymptomatic meningiomas, especially those with an initial diameter < 2 cm.

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Trigeminal schwannomas: experience with 57 cases and a review of the literature

Neurosurg Rev. DOI 10.1007/s10143-010-0289-y

Trigeminal schwannoma is a mostly benign tumor that can be cured by complete resection. Over the last few decades, several pioneers have developed surgical approaches enabling the total removal of such tumors.

We analyzed 57 patients who underwent radical surgery, including 45 patients who underwent skull base surgery as their initial treatment, for removal of trigeminal schwannomas. Here, we report the surgical management of these cases. Since 1990, all such patients have been treated using three main types of middle fossa skull base approaches, which minimize the exposure of the brain: the anterior transpetrosal approach, subtemporal interdural approach (Dolenc), or a combination of these approaches.

Before 1990, total tumor removal was achieved in only three of eight patients (38%). After 1990, the tumors were totally removed in 43 patients (90%) and were nearly completely removed in an additional three patients (6%). Among the patients who underwent skull base surgery as their initial treatment, a complete resection was achieved in 93% (42/45 patients) of the cases. However, total surgical removal after surgery and Gamma knife surgery was very difficult because of dense adhesions to the brain stem and cranial nerves. No surgery-related mortalities occurred in this series, and the individual KPS scores were more than 90% among the patients who underwent skull base surgery. No recurrences requiring additional surgery have occurred after an average follow-up period of 4.9 years.

Most of the trigeminal schwannomas could be removed totally and safely during a single operation after the introduction of skull base surgery. Therefore, radiosurgery should not be applied as the treatment of first choice for younger patients. A correct anatomical knowledge is critical for minimizing brain exposure and avoiding surgical complications.

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Clival chordomas: clinical management, results, and complications in 71 patients

J Neurosurg 113:1059–1071, 2010. DOI: 10.3171/2009.9.JNS08596

Chordomas are rare malignant neoplasms arising predominantly at the sacrum and skull base. They are uniformly lethal unless treated with aggressive resection and proton beam irradiation. The authors present results of the surgical management of a large number of patients with clivus chordomas. Factors that influence the surgeon’s ability to achieve radical tumor resection are also evaluated.

Methods. Between 1991 and 2005, 71 patients with clivus chordomas underwent surgery. The average followup was 66 months (median 60 months, range 3–189 months). Sixty-five patients had complete records that were analyzed in the present report. Thirty-five percent of them had undergone surgery before being treated by the authors. They were evaluated with MR imaging and CT scanning and underwent surgery utilizing a variety of skull base techniques aimed at achieving radical excision. Many also underwent postoperative radiation, usually in the form of proton beam therapy. The patients were followed up with serial imaging at regular intervals as well as with neurological evaluation.

Results. Radical tumor resection was achieved in 58% of the group. The overall 5-year survival rate was 75%. Radical resection had a positive impact on survival. The ability to achieve radical resection was dependent on the preoperative tumor volume and the number of anatomical areas involved by the tumor. Cranial nerve impairment and CSF leakage were the most frequent postoperative complications.

Conclusions. Radical excision is the ideal surgical goal in the treatment of clival chordomas and can be achieved with reasonable risks. Several different surgical approaches may be necessary to accomplish this.

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Surgical management of giant pediatric craniopharyngiomas

J Neurosurg Pediatrics 6:000–000, 2010.DOI: 10.3171/2010.8.PEDS09385

Prior work by the authors’ group and reports of other authors suggest worse functional outcomes and decreased survival in children with larger craniopharyngiomas. The purpose of this study was to assess the oncological, endocrinological, and functional outcomes in children who underwent radical resection of giant craniopharyngiomas (defined as 5 cm or greater in largest diameter).

Methods. Between 1986 and 2006, 26 children under the age of 18 (14 boys, 12 girls; mean age 10.5 years) underwent radical resection of giant craniopharyngiomas performed by the senior author. Data were retrospectively collected to assess the outcome of surgical treatment.

Results. Twenty (77%) of 26 patients underwent gross-total resection (GTR) confirmed by intraoperative inspection and postoperative imaging. All primary tumors (17 of 17) and 3 (33%) of 9 recurrent tumors were treated with GTR. There was no operative mortality, and 18 of 26 patients (69%) were alive at a mean follow-up of 8.9 years (median 9.3 years). Disease control was achieved in 21 (84%) of the 25 patients followed up for more than 6 months and was more successful in patients who underwent GTR (95%) than in those who underwent STR (50%, p = 0.03). New-onset diabetes insipidus (DI) occurred in 63.2% of patients (73% of patients had DI postoperatively). New or worsened deficits in visual acuity and visual fields occurred in 16% and 28%, respectively, of the 25 patients for whom postoperative visual data were available. Five patients (19%) experienced significant, permanent neurological deficits, and 5 (19%) had mild to moderate deficits. New or worsened hypothalamic disturbance occurred in 35% and 22% of patients, respectively, but obesity developed in only 15%.

Conclusions. In this retrospective series, radical resection of giant craniopharyngiomas in children was found to lead to excellent rates of disease control with acceptable or good functional outcomes but slightly higher rates of neurological complications compared with rates in patients with smaller tumors. Radical resection is less successful in recurrent tumors that reach very large sizes, especially previously irradiated tumors, with resultant diminished survival.

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Trigeminal neurinomas with extracranial extension: analysis of 28 surgically treated cases

J Neurosurg 113:1079–1084, 2010.DOI: 10.3171/2009.10.JNS091149

The object of this paper was to review the authors’ experience with 28 cases of trigeminal neurinomas having an extracranial extension.

Methods. The authors analyzed 28 cases of trigeminal neurinoma in which there was an extracranial extension of the tumor. All patients were treated in their department between the years 1989 and 2009.

Results. There was tumor extension along the ophthalmic division of the nerve in 4 cases, along the maxillary division in 5, and along the mandibular division in 13. In 6 tumors there was diffuse extracranial extension and the exact extracranial division of nerve involvement could not be ascertained. In 10 cases, the tumor had a multicompartmental location—in the posterior fossa, the middle fossa, and the extracranial compartment. Tingling paraesthesiae, numbness, and diffuse pain in the distribution of the trigeminal nerve were common symptoms and were present in 90% of patients. The extracranial component had a well-defined perineural/meningeal membrane cover that was continuous with the middle fossa dura mater and isolated the tumor tissue from the adjoining critical structures. In 7 out of 10 cases, even the posterior fossa component of the tumor was entirely “interdural” (within the confines of the dura). The maximum dimension of the tumor was > 4 cm in 22 cases. A limited “transcranial” approach with (12 cases) or without (16 cases) zygomatic osteotomy was found suitable for resection of these tumors. In 4 cases a lateral orbitotomy was performed. Total tumor resection was performed in 20 cases and partial resection in 8. The duration of follow-up ranged from 6 months to 19 years. Two patients required additional surgery for symptomatic recurrence.

Conclusions. Extracranial extensions of trigeminal neurinomas have a well-defined meningeal covering. In most cases resection was performed via a minimally invasive cranial avenue (a “reverse skull base approach”). Radical resection was associated with an excellent long-term outcome.

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Utility of diffusion tensor-imaged (DTI) motor fiber tracking for the resection of intracranial tumors near the corticospinal tract

Acta Neurochir. DOI 10.1007/s00701-010-0817-0

Treatment of intracranial tumors near the corticospinal tract remains a surgical challenge. Several technical tools to map and monitor the motor tract have been implemented. The present study aimed to assess the utility of diffusion tensor imaging (DTI) fiber tracking in the surgical treatment of motor eloquent tumors at our institution.

Methods Patients operated for intracranial tumors close to the motor tract with the use of intraoperative image guidance including DTI fiber tracking of the corticospinal tract and intraoperative motor evoked potential (MEP) monitoring were analyzed. The intraoperative utility of fiber tracking data was analyzed. Furthermore, preoperative MRI scans with and without motor fiber tracking were reevaluated post hoc for tumor relation to the motor tract, estimated resectability, and best approach. Thereby, the utility of fiber tracking in surgical planning was assessed.

Results Nineteen patients were analyzed. The estimation of tumor localization in relation to the motor tract and of resectability was not influenced by fiber tracking in any of the cases. Only in one single case did evaluating surgeons change their surgical approach after the addition of the fiber tracking data. In all cases, fiber tracking included in image guidance did not change the intraoperative strategy, while MEP monitoring did.

Conclusions DTI fiber tracking did not influence the surgical planning or the intraoperative course. However, it is still used at our institution due to its ease in acquisition and its potential impact in a larger series. Furthermore, more experience with this technique is required to lead to a technical improvement.

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