Category: Oncology

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Removal of giant extraforaminal dumbbell tumors of cervical spine

The Spine Journal 9 (2009) 822–829. doi:10.1016/j.spinee.2009.06.023

Removal of cervical dumbbell tumors can be particularly challenging because of unique exposure requirements and proximity of the vertebral artery (VA). There are no reports describing the treatment of giant cervical spine dumbbell tumors (CSDTs).

PURPOSE: To introduce an extensive posterolateral approach to CSDTs involving total lateral mass resection and laminectomy.

STUDY DESIGN: Prospective study of all the patients with multilevel CSDTs treated by  this new procedure between December 2002 and March 2006.

PATIENT SAMPLE: Sixteen patients (3 men and 13 women) with CSDTs underwent the procedure we describe. The follow-up periods ranged from 9 to 51 months (average 9 months). Average age at surgery was 45 years (range 23–68 years).

OUTCOME MEASURES: Axial symptoms and Japanese Orthopedic Association scores were recorded. Pre- and postoperative ranges of neck motion were measured on lateral flexion and extension radiographs.

METHODS: After making a midline incision, we preferred exposing the extraforaminal component of the tumor before performing a semilaminectomy and lateral mass resection. Any lateral extensión of a tumor can be attained by detachment of the adjacent three or more segments of the lateral mass muscle insertion. The most lateral portion can be separated beneath the tumor’s superficial muscle flap, and then when the tumor is retracted medially, the whole portion of the lateral component can be totally exposed. We then performed total lateral mass resection and laminectomy to expose the tumor at the foramina and cervical canal.

RESULTS: We were able to completely resect the tumors in every patient. The average duration of surgery was 150 minutes. Blood loss was minimal (average 400 mL). All patients were monitores for a minimum of 9 months (range 9–51 months; mean 28 months). The follow-up period was uneventful, and no patients developed spinal instability.

CONCLUSIONS: Extensive posterolateral exposure enables surgeons to reach the lateralmost portion of CSDTs and also facilitates septation of the VA and resection of vertebral body encroachment of the tumor.


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Improving the prognosis for patients with glioblastoma: the rationale for targeting Src

J Neurooncol (2009) 95:151–163 DOI 10.1007/s11060-009-9916-2

Glioblastoma is the most common and aggressive form of primary brain tumor. The prognosis for patients diagnosed with glioblastoma is poor, with a median survival of 12–14 months and a 5-year survival rate of <5%. The upfront standard treatment for patients with newly diagnosed glioblastoma, consisting of surgery followed by chemotherapy combined with radiotherapy, provides only short-term survival benefits. Recurrent glioblastoma is an extremely challenging therapeutic setting because of the aggressive and resistant nature of the tumor. A set of key molecular targets in oncology is the Src family of non-receptor protein kinases. Dysregulated signaling via the Src kinases has been shown to underlie glioma-related proliferation, angiogenesis, migration, and survival. Here we review the biologic role of Src in malignant glioma and discuss key preclinical studies demonstrating the potential utility of inhibiting Src in glioma. Proof of clinical benefit is forthcoming from the first clinical studies involving the newest generation of small molecule Src inhibitors currently in clinical trials for recurrent glioblastoma. Blocking Src alone will likely not translate into a significant clinical benefit; thus, strategies for combining Src inhibitors with potential synergistic therapeutic modalities will be discussed. This review focus on dasatinib, the most advanced Src inhibitor being tested in glioblastoma, which is currently in phase I/II trials in this setting.


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Foramen magnum meningiomas: experiences in 114 patients

Surgical Neurology 72 (2009) 376–382. doi:10.1016/j.surneu.2009.05.006

Background: Although there has been great development in the anatomical understanding and operative techniques for skull base tumors, controversy still exists regarding the optimal surgical strategies for the FMMs. We report clinical and radiologic features as well as the surgical findings and outcome for patients with FMM treated at our institution over the last 15 years.
Methods: We reviewed 114 consecutive cases of FMM operated between May 1993 and June 2008 in the neurosurgery department at Beijing Tiantan Hospital.
Results: There were 68 female and 46 male patients (mean age, 52.3 years; range, 28-76 years). Foramen magnum meningiomas were classified as anterior (80 cases), anterolateral (24 cases), and posterolateral (10 cases). Mean duration of symptoms was 11.7 months (ranging from 1.5 to 240 months). Cervico-occipital pain (80.7%) and headache and dizziness (42.1%) were the most common presenting symptoms. The preoperative KPS was 72.5 ± 8.3. Mean maximum diameter of the tumors on MRI was 3.35 cm (range, 1.5-4.7 cm). Posterior midline approach was performed in 10 cases, far-lateral retrocondylar approach in 97 cases, and extended far-lateral approach in 7 cases. Gross total resection was achieved in 86.0% of patients and subtotal resection in 14.0%. Surgical mortality was 1.8%. Follow-up data were available for 93 patients, with a mean follow-up of 90.3 months (range, 1-180 months), of which 59 (63.4%) lived a normal life (KPS, 80-100).
Conclusion: Our experience suggests that most anterior and anterolateral FMMs can be completely resected by a far-lateral retrocondylar approach without resection of the occipital condyle. Complete resection of the tumor should be attempted at the first operation. Postoperative management of FMM is important for the prognosis.

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Risk of stereotactic biopsy in eloquent brain regions

J Neurosurg 111:820–824, 2009. DOI: 10.3171/2009.3.JNS081695

Object. Frameless stereotactic biopsy has been shown in multiple studies to be a safe and effective tool for the diagnosis of brain lesions. However, no study has directly evaluated its safety in lesions located in eloquent regions in comparison with noneloquent locations. In this study, the authors determine whether an increased risk of neurological decline is associated with biopsy of lesions in eloquent regions of the brain.
Methods. Medical records, including imaging studies, were reviewed for 284 cases in which frameless stereotactic biopsy procedures were performed by 19 neurosurgeons at 7 institutions between January 2000 and December 2006. Lesion location was classified as eloquent or noneloquent in each patient. The incidence of neurological decline was calculated for each group.
Results. During the study period, 160 of the 284 biopsies predominately involved eloquent regions of the brain. In evaluation of the complication rate with respect to biopsy site, neurological decline occurred in 9 (5.6%) of 160 biopsies in eloquent brain areas and 10 (8.1%) of 124 biopsies in noneloquent regions; this difference was not statistically significant (p = 0.416). A higher number of needle passes was associated with the presence of a postoperative hemorrhage at the biopsy site, although not with a change in the result of neurological examination.
Conclusions. Frameless stereotactic biopsy of lesions located in eloquent brain regions is as safe and effective as biopsy of lesions in noneloquent regions. Therefore, with careful planning, frameless stereotactic biopsy remains a valuable and safe tool for diagnosis of brain lesions, independent of lesion location.

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Meningiomas of the ventral foramen magnum and lowerclivus

Acta Neurochir DOI 10.1007/s00701-009-0511-2

Purpose To identify an appropriate surgical approach for meningiomas of the foramen magnum and lower clivus and determine the factors influencing the surgical outcomes. Method The study involved 23 patients with foramen magnum or lower clival meningiomas (8 men, 15 women; average age, 56 years; range, 26–70 years) treated at Keio University Hospital between 1991 and 2008. Their clinical data were retrospectively reviewed with regard to the surgical approaches and outcomes. The average follow-up duration was 42.8 months, the mean tumour size, 25.9 mm (range, 12.0–50.0 mm).

Findings The tumours most commonly originated in the anterolateral rim of the foramen magnum. In 12 cases with lateral compression of the brain stem, the tumours were resected via the suboccipital approach with C1 laminectomy. The transcondylar approach was adopted in 11 cases where the tumour was located on the anterior rim of the foramen magnum. Four patients required epidural drilling of the jugular tubercle. Gross total resection was achieved in 15 cases (62.5%), the resection rate being lowest in cases of tumour extension to the lower clivus. The transient and permanent morbidity rates were 30.4% and 17.4%, respectively. Logistic regression analysis revealed that extension to the lower clivus (noted in 6 of 23 patients) was a statistically significant, independent factor influencing the permanent morbidity rate (p = 0.005)

Conclusions Selecting an appropriate surgical approach considering the tumour location resulted in a good surgical outcome. However, tumour extension to the lower clivus strongly influenced the morbidity and tumour radicality

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Bevacizumab Alone and in Combination With Irinotecan in Recurrent Glioblastoma

Journal of Clinical Oncology. DOI:10.1200/JCO.2008.19.8721

Purpose

We evaluated the efficacy of bevacizumab (Avastin TM), alone and in combination with irinotecan, in patients with recurrent glioblastoma in a phase II, multicenter, open-label, noncomparative trial.

Patients and Methods

One hundred sixty-seven patients were randomly assigned to receive bevacizumab 10 mg/kg alone or in combination with irinotecan 340 mg/m2 or 125 mg/m2 (with or without concomitant enzyme-inducing antiepileptic drugs, respectively) once every 2 weeks. Primary end points were 6-month progression-free survival and objective response rate, as determined by independent radiology review. Secondary end points included safety and overall survival.

Results

In the bevacizumab-alone and the bevacizumab-plus-irinotecan groups, estimated 6-month progression-free survival rates were 42.6% and 50.3%, respectively; objective response rates were 28.2% and 37.8%, respectively; and median overall survival times were 9.2 months and 8.7 months, respectively. There was a trend for patients who were taking corticosteroids at baseline to take stable or decreasing doses over time. Of the patients treated with bevacizumab alone or bevacizumab plus irinotecan, 46.4% and 65.8%, respectively, experienced grade  3 adverse events, the most common of which were hypertension (8.3%) and convulsion (6.0%) in the bevacizumab-alone group and convulsion (13.9%), neutropenia (8.9%), and fatigue (8.9%) in the bevacizumab-plus-irinotecan group. Intracranial hemorrhage was noted in two patients (2.4%) in the bevacizumab-alone group (grade 1) and in three patients (3.8%) patients in the bevacizumabplus-irinotecan group (grades 1, 2, and 4, respectively).

Conclusion

Bevacizumab, alone or in combination with irinotecan, was well tolerated and active in recurrent glioblastoma.

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Prognostic factors in intramedullary astrocytomas

Eur Spine J (2009) 18:1397–1422 DOI 10.1007/s00586-009-1076-8

Astrocytomas affect a significant portion of patients with intramedullary tumors. These infiltratively growing tumors are treated by a variety of methods—biopsy and decompressive surgery, maximal safe resection, adju- vant oncological therapy. Also, numerous prognostic factors are reported in the literature. Better understanding of factors that influence prognosis may help in treatment planning with the goal of prolonging survival. We have thus undertaken an extensive literature review in order to define factors affecting prognosis. A total of 38 articles were studied. Only tumor grade was consistently reported as the major factor affecting prognosis. The influence of other clinical factors (age, gender, history length, functional status, tumor location or extent, syrinx or cyst presence) can be speculated upon, but cannot be assessed adequately from the available literature. For both low- and high-grade (HG) astrocytomas, maximal safe tumor resection should be the primary treatment objective but is often not feasible in contrast to other intramedullary and spinal neoplasms. Since the biological nature of spinal cord HG glioma is identical to that of the brain, the same treatment algorithm of maximal safe resection followed by concomitant radio and chemotherapy would be sensible to implement.

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Neurosurgical management of intractable rolandic epilepsy in children: role of resection in eloquent cortex

J Neurosurg Pediatrics 4:199-216, 2009. DOI: 10.3171/2009.3.PEDS08459

Object. The authors undertook this study to review their experience with cortical resections in the rolandic region in children with intractable  epilepsy.
Methods. The authors retrospectively reviewed the medical records obtained in 22 children with intractable epilepsy arising from the rolandic region. All patients underwent preoperative   electroencephalography (EEG), MRimaging, prolonged video-EEG recordings, functional MR imaging, magnetoencephalography, and in some instances PET/SPECT studies. In 21 patients invasive subdural grid and depth electrode monitoring was performed. Resection of the epileptogenic zones in the rolandic region was undertaken in all cases. Seizure outcome was graded according to the Engel classification. Functional outcome was determined using validated outcome scores.
Results. There were 10 girls and 12 boys, whose mean age at seizure onset was 3.2 years. The mean age at surgery was 10 years. Seizure duration prior to surgery was a mean of 7.4 years. Nine patients had preoperative hemiparesis.
Neuropsychological testing revealed impairment in some domains in 19 patients in whom evaluation was possible. Magnetic resonance imaging abnormalities were identified in 19 patients. Magnetoencephalography was performed in all patients and showed perirolandic spike clusters on the affected side in 20 patients. The mean duration of invasive monitoring was 4.2 days. The mean number of seizures during the period of invasive monitoring was 17. All patients underwent resection that involved primary motor and/or sensory cortex. The most common pathological entity encountered was cortical dysplasia, in 13 children. Immediately postoperatively, 20 patients had differing degrees of hemiparesis, from mild to severe. The hemiparesis improved in all affected patients by 3–6 months postoperatively. With a mean follow-up of 4.1 years (minimum 2 years), seizure outcome in 14 children (64%) was Engel Class I and seizure outcome in 4 (18%) was Engel Class II. In this series, seizure outcome following perirolandic resection was intimately related to the child’s age at the time of surgery. By univariate logistic regression analysis, age at surgery was a statistically significant factor predicting seizure outcome (p < 0.024).
Conclusions. Resection of rolandic cortex for intractable epilepsy is possible with expected morbidity. Accurate mapping of regions of functional cortex and epileptogenic zones may lead to improved seizure outcome in children with intractable rolandic epilepsy. It is important to counsel patients and families preoperatively to prepare them for possible worsened functional outcome involving motor, sensory and/or language pathways.

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Endoscopic treatment of third ventricular colloid cysts

Neurosurg Rev (2009) 32:395–402. DOI 10.1007/s10143-009-0208-2

The surgical treatment of colloid cysts has been traditionally difficult with high rate of postoperative complications. The variety of surgical options reflects the technical difficulty in removing these benign lesions with low morbidity. Microsurgical removal has for years been considered the “gold standard” of treatment, with the use of either a transcortical–transventricular or a transcallosal approach. Neuroendoscopic management is emerging as a safe, effective alternative to microsurgery. The present review discusses the role

of endoscopy in the surgical treatment of third ventricular colloid cysts focusing on some factors, which might influence the outcome. The results have been presented from the literature and supplemented by the results of treating ten personal cases of third ventricular colloid cysts who were operated endoscopically in the Neurosurgical Department, Cairo University. This study aims at evaluating the endoscopic approach as a surgical line of treatment in the management of third ventricular colloid cysts and to see if it has already become superior over microsurgery.

Conclusions: Endoscopic approach to third ventricular colloid cysts is a minimally invasive procedure, which achieves both total evacuation of the cyst and at least near-total resection of the cyst wall with a low surgical morbidity. The available results document less radical excisions as compared to microsurgical group; this is counterbalanced by the lower incidence of complications and shorter operative, hospitalization, and rehabilitation time in the endoscopic group. This conclusion makes endoscopy an alternative and not a better choice than microsurgery. Consequently, the ongoing debate between both procedures remains unresolved. This dispute will become resolved only when long-term studies (mean follow-up for 10 years or greater) are available for a substantial number of patients who have undergone endoscopic resection.

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Do children and adults differ in survival from medulloblastoma? A study from the SEER registry

J Neurooncol (2009) 95:81–85 DOI 10.1007/s11060-009-9894-4

Studies investigating whether adults have diminished survival from medulloblastoma (MB) compared with children have yielded conflicting results. We sought to determine in a population-based registry whether adults and children with MB differ in survival, and to examine whether dissimilar use of chemotherapy might contribute to any disparity.

1,226 MB subjects were identified using the Surveillance Epidemiology and End Results (SEER-9) registry (1973–2002) and survival analysis performed. MB was defined strictly to exclude non-cerebellar primitive neuro- ectodermal tumors. Patients were stratified by age at diagnosis: <3 years (infants), 3–17 years (children) and >18 years (adults). Because the SEER-9 registry lacks treatment data, a subset of 142 patients were identified using the San Francisco-Oakland SEER registry (1988–2003) and additional analyses performed.

There was no significant difference in survival between children and adults with MB in either the SEER-9 (P = 0.17) or SFO (P = 0.89) cohorts but infants fared worse compared to both children (P < 0.01) and adults (P < 0.01). In the SFO sample, children and adults who received chemotherapy plus radiation therapy (XRT) did not differ in survival. Among patients treated with XRT alone, children showed increased survival (P = 0.04) compared with adults. Children and adults with MB do not differ with respect to overall survival, yet infants fare significantly worse.

For children and adults with MB treated with both XRT and chemotherapy, we could not demonstrate a survival difference. Similar outcomes between adult and childhood MB may justify inclusion of adults in pediatric cooperative trials for MB.

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Trigeminal neurinomas: clinical features and surgical experience in 84 patients

Neurosurg Rev (2009) 32:435–444.DOI:10.1007/s10143-009-0210-8

Trigeminal neurinomas are the second most common intracranial neurinomas next to the vestibular neurinomas. Eighty-four patients with trigeminal neurinomas were treated between 2003 and 2007. There were 40 women and 44 men (mean age 43 years). The most frequent symptoms were headache or numbness of the ipsilateral hemiface. There were 24 type A, nine type B, 45 type C, and six type D tumors. Dextroscope virtual reality technology was used for preoperative planning in recent eight cases. Gross total resection was achieved in 63 patients. We found that the major impediments to complete removal were adherent to the brainstem and skull base vascular structure, the frontotemporal approach with zygomatic or orbitozygomatic osteotomy or subtemporal approach could offer excellent exposure of the middle fossa and access to the posterior fossa, and Dextroscope virtual reality technology was a very useful tool to identify surgical and anatomic nuances and enhance preoperative planning in trigeminal neurinomas resection.

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Tentorial meningiomas: operative nuances and perioperative management dilemmas

Acta Neurochir (2009) 151:1037–1051.DOI 10.1007/s00701-009-0421-3

Tentorial meningiomas (TM), comprising approximately 3–6% of all intracranial meningiomas, are complex entities with an intricate relationship to surrounding structures and require multiple surgical approaches. In the present study, the rationale for deciding the approaches for TMs and the perioperative management dilemmas were evaluated.
Methods  Thirty-seven patients (28 primary [supratentorial (2), infratentorial (20) and both (6)] and nine complex [cerebellopontine (CP) angle (5) and petroclival (4)] TM) underwent surgery using the occipital transtentorial, supracerebellar infratentorial, subtemporal transtentorial, bioccipital suboccipital, midline suboccipital, retrosigmoid, and combined pre and retrosigmoid approaches. The extent of excision was categorized according to Simpson’s grade.
Results  Simpson’s grade of excision was I in six, II in 11, III in nine and IV in 11 patients, respectively. Follow-up assessment (2 months to 9 years) in 27 patients (72.9%) showed that 23 patients returned to their previous activity level with either no or minimal symptoms, three returned to previous activity level with major cranial nerve palsy, and one patient required permanent assistance. One patient had recurrence and four others underwent resurgery for residual tumor. Two patients with petroclival lesions died due to aspiration pneumonitis and meningitis, respectively; one with CP angle TM presented in a poor general condition and expired following emergency ventriculoperitoneal shunt and subsequent definite surgery. Pseudomeningocele, cerebrospinal fluid leak, and cranial nerve palsy were the major morbidities.
Conclusions  Classifying TM into medial and lateral, supra and infratentorial groups helps in deciding an appropriate and safe approach. Meticulously preserving venous sinuses is important since the risk of venous infarction cannot be predicted even with radiological good venous collaterization and apparent venous sinus blockade by tumor. Laterally situated tumors carry a better prognosis when compared to the medially situated ones. Leaving a small residual tumor in an effort to preserve important neurovascular structures does not obviate the expectation of a good long-term prognosis with minimal morbidity and low recurrence rates.

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Adult craniopharyngiomas: surgical results with a special focus on endocrinological outcomes and recurrence according to pituitary stalk preservation

DOI: 10.3171/2008.10.JNS0880

The aim of this study was to evaluate the results of surgical treatment of adult craniopharyngioma with a special focus on the endocrinological outcomes and tumor recurrence in cases of pituitary preservation.

Methods: Between 1993 and February 2008, 41 patients underwent 47 surgical procedures for craniopharyngioma. The male/female ratio was 26:15 and the median age was 45.8 years (range 17–65 years). The median follow-up period was 10.56 years (range 6.2–14.9 years). Patients presented with visual disturbance before 30 (63.8%) of 47 procedures and with endocrinological disturbance before 12 (26%) procedures. Surgery was performed via a subfrontal/pterional approach in 31 procedures (66%), bifrontal interhemispheric in 6 (12.8%), transcallosal/transventricular in 5 (10.6%), combined in 1 (2.1%), and transsphenoidal in 4 (8.5%). The tumor was totally removed in 36 procedures (76.6%), subtotally in 10 (21.3%), and partially in 1 (2.1%).

Results: Postoperatively, the rates of visual improvement and aggravation were 50 and 33.3%, respectively. Of 24 patients in whom the pituitary stalk was preserved, complete hormone replacement was needed in 14 (58.3%), partial replacement in 2 (8.3%), and no replacement in 8 (33.3%). The rate of tumor recurrence was 24.4%. The recurrence-free survival rate was significantly different between patients in whom complete tumor resection was accomplished and those in whom tumor resection was incomplete. Stalk preservation did not affect the recurrence-free survival rate. The morbidity and mortality rates were 8.5 and 2.1%, respectively.

Conclusions: The pituitary stalk must be preserved with maximal tumor resection whenever possible to increase the chance of intact anterior pituitary function being maintained. The results of the present study show that pituitary stalk preservation may not be related to increased recurrence rates.

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Residual nonfunctioning pituitary adenomas: prognostic value of MIB-1 labeling index for tumor progression

J Neurosurg 111 (3)563-571. DOI: 10.3171/2008.4.17517

In residual nonfunctioning pituitary adenomas, reliable prognostic parameters indicating probability of tumor progression are needed. The Ki 67 expression/MIB-1 labeling index (LI) is considered to be a promising candidate factor. The aim in the present study was to analyze the clinical usefulness of MIB-1 LI for prognosis of tumor progression.

Methods: The authors studied a cohort of 92 patients with nonfunctioning pituitary adenomas. Based on sequential postoperative MR images, patients were classified as tumor free (51 patients) or as harboring residual tumor (41 individuals). The residual tumor group was further subdivided in groups with stable residual tumors (14 patients) or progressive residual tumors (27 patients). The MIB-1 LI was assessed in tumor specimens obtained in all patients, and statistical comparisons of MIB-1 LI of the various subgroups were performed.

Results: The authors found no significant difference of MIB-1 LI in the residual tumor group compared with the tumor-free group. However, MIB-1 LI was significantly higher in the progressive residual tumor group, compared with the stable residual tumor group. Additionally, the time period to second surgery was significantly shorter in residual adenomas showing an MIB-1 LI > 3%.

Conclusions: The data indicate that MIB-1 LI in nonfunctioning pituitary adenomas is a clinically useful prognostic parameter indicating probability of progression of postoperative tumor remnants. The MIB-1 LI may be helpful in decisions of postoperative disease management (for example, frequency of radiographic intervals, planning for reoperation, radiotherapy, and/or radiosurgery).

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Biopsy versus resection in the management of malignant gliomas

DOI: 10.3171/2009.7.JNS09758

The aim of this study was to answer the question whether quality of life and progression-free and overall survival are increased in adults with supratentorial malignant glioma who are treated with cytoreductive resection as compared with those who only undergo biopsy.

Methods. A literature search of the electronic databases MEDLINE, EMBASE, and CENTRAL was performed to identify relevant studies published before May 2008. Hand-searching of reference lists of the identified studies and relevant review articles was also performed. A study was considered eligible, regardless of study design (prospective or retrospective), if: 1) quality of life and/or progression-free and/or overall survival was compared among adult patients undergoing biopsy or resection, and 2) patient age and Karnofsky Performance Scale scores were not significantly different among the 2 groups compared.

Results. One randomized controlled trial and 4 retrospective studies (involving a total of 1111 patients) were found eligible for this systematic review. A meta-analysis of the eligible studies demonstrated a significant increase in overall survival in the patients treated with resection instead of biopsy (hazard ratio 0.61, 95% CI 0.52–0.71, p <0.0001, fixed-effect model). Although statistical pooling was not feasible, the available data suggest that quality of life was increased in patients treated with resection rather than biopsy, while there did not seem to be any significant difference in progression-free survival between the 2 groups.

Conclusions. Based on the best available evidence, it appears that cytoreductive resection in adults with supratentorial malignant glioma is associated with improved overall survival as compared with biopsy. However, welldesigned prospective studies are needed for more solid conclusions to be drawn.

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Determinants of postoperative visual recovery in suprasellar meningiomas

Acta Neurochirurgica DOI: 10.1007/s00701-009-0492-1

Suprasellar meningiomas usually present with visual deterioration, including decreased visual acuity and/or visual field defects. Suprasellar meningiomas have a close relationship with the optic apparatus, arteries of the anterior circulation, pituitary stalk and hypothalamus, which makes safe surgical resection a challenge especially with dissection around an already compromised optic apparatus. In this report 21 patients operated on for a suprasellar meningioma over a 4-year period are reviewed. Postoperative outcome and visual recovery are evaluated, including analysis of its determinants.
Methods Over a 4-year period (2002–2006), patients surgically treated for suprasellar meningiomas were included in this retrospective study. All tumors were located at the tuberculum sellae and diaphragma sellae dura. Clinical and neuro-ophthalmological examinations, imaging studies, endocrinological evaluation and follow-up data were reviewed retrospectively. The influence of patient age, sex, duration of symptoms, extent of visual impairment, tumor size , extent into optic canal, consistency, operative respectability were analyzed as potential prognostic factors for postoperative visual outcome.
Results Twenty-one patients were included in this retrospective study. Ages ranged from 25 to 65 years (mean: 43 years). All patients had visual acuity loss and visual field defects. Symptom duration ranged from 2 to 36 months (mean: 17 months). Tumor removal was complete in 17 patients, and subtotal resection was performed in four patients. There was one case of postoperative mortality. The follow-up duration ranged from 24 to 48 months (mean: 28 months). At the last follow-up 12 patients (60%) had achieved visual improvement, whereas vision was unchanged in eight patients (40%). None of the patients had visual deterioration during their follow-up. A univariate analysis of clinical and surgical parameters thought to be related to visual outcome showed that the duration of symptoms, preoperative visual status, tumor size and adherence to the internal carotid arteries and/or anterior cerebral artery had a significant impact on visual outcome.

Conclusion The extent and duration of visual symptoms, size of the tumor and vascular adherence were prognostic factors affecting visual recovery after microsurgical resection of suprasellar meningiomas.

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Survival and prognostic factors in a series of adults with medulloblastomas

J Neurosurg 111 (Sept 2009) DOI: 10.3171/2009.1.JNS081004

In this article, the authors report their experience in the management of adult patients with medulloblastoma at their institution to identify prognostic factors important for survival and disease control.

Between 1977 and 2005, 27 patients who were ≥ 16 years old and had medulloblastoma were treated consecutively. There were 16 women and 11 men with a median age of 21 years (range 16–54 years). Gross-total resection was performed in 21 patients, subtotal (≥ 90%) in 2, incomplete in 1, and biopsy in 3 patients. Six patients had the desmoplastic variant, and 21 patients presented with classic medulloblastoma. Staging according to the Chang classification showed 4 patients with tumors invading the brainstem (2 with Stage T3b and 2 with Stage T4), 3 patients with metastases (2 with Stage M2 and 1 with Stage M3), and 1 patient in whom the stage was unknown (Stage MX) who died 10 days postoperatively. Twenty patients were assigned to the standard-risk group and 7 to the high-risk group. All patients except the one whose status was classified as Stage MX underwent craniospinal radiotherapy at our institution. Seven patients received chemotherapy before radiotherapy.

The 5- and 10-year overall survival rates for the present study were 81 and 62%, respectively. The median overall survival time was 17.7 years. The 5- and 10-year event-free survival rates were 72 and 57%, respectively. The median event-free survival time was 17.9 years. Univariate analysis showed that survival was significantly correlated with sex (women had a better prognosis than men) and M stage (patients without metastases had a better outcome). Patient age, duration of symptoms, Karnofsky Performance Scale score at presentation, hydrocephalus, tumor location, brainstem invasion, extent of resection, histological subtype, preradiotherapy chemotherapy, risk group, and period of presentation were not significant variables. Multivariate analysis identified sex and M stage as well as the period of presentation as independent prognostic factors for overall and event-free survival times. Eleven patients suffered tumor recurrence within a median time of 4.2 years. The posterior fossa was not the most common site of recurrence, and delayed recurrence was not rare. All patients in whom the tumor recurred have died despite aggressive treatments. The median survival time after diagnosis of recurrence was 2.5 years. Questionnaires on quality of life and cognition showed high scores in favor of limited negative effects in the perception of mental and physical health after treatment. The authors observed 1 supposed second malignancy (thyroid carcinoma) and no evidence of pituitary dysfunction.

Conclusions: Long-term survival is possible in adults treated for medulloblastoma. Although rare, metastasis seeding at presentation is a poor prognostic factor. The possibility of delayed recurrence necessitates close follow-up of all patients. Tumor recurrences should be treated with aggressive therapies as some patients may have sustained response. Adjuvant chemotherapy should be given to high-risk patients, but its role in reducing recurrences, particularly distant ones, remains unclear in the standard-risk group.

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Treatment of Medulloblastoma with Hedgehog Pathway Inhibitor GDC-0449

Published at www.nejm.org September 2, 2009 (10.1056/NEJMoa0902903)

Medulloblastoma is the most common malignant brain tumor in children. Aberrant activation of the hedgehog signaling pathway is strongly implicated in the development of some cases of medulloblastoma. A 26-year-old man with metastatic medulloblastoma that was refractory to multiple therapies was treated with a novel hedgehog pathwayinhibitor, GDC-0449; treatment resulted in rapid (although transient) regression of the tumor and reduction of symptoms. Molecular analyses of tumor specimens obtained before treatment suggested that there was activation of the hedgehog pathway, with loss of heterozygosity and somatic mutation of the gene encoding patched homologue 1 (PTCH1), a key negative regulator of hedgehog signaling.

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Gamma knife radiosurgery for the treatment of glomus jugulare tumors

Journal of Neuro-Oncology. doi:10.1007/s11060-009-0002-6

The treatment of glomus jugulare tumors represents a challenge for the neurosurgeon, since they invade major vessels and compress critical cranial nerves, resulting in significant morbidity from tumor resection. Among alternative and complementary treatment options, gamma knife radiosurgery is a less invasive procedure and may provide better protection of vital structures. This study aimed to evaluate the efficacy and long-term outcomes of gamma knife surgery in the treatment of these tumors in a large series with the longest follow-up period compared with previous reports. A total of 18 patients with glomus jugulare tumors that underwent gamma knife radiosurgery (GKS) were included. Eleven patients had a history of previous microsurgical treatment. The mean marginal radiation dose was 15.6 Gy (median 15 Gy, range 13–20 Gy). Patients were followed for a mean period of 52.7 months (median 41.5 months); the effect of gamma knife radiosurgery was evaluated using magnetic resonance (MR) images. Based on the last MR images, tumor control could be achieved in 17 out of 18 patients (94.4%). No complications such as radiation-induced peritumoral edema or radiation necrosis occurred. Neurological follow-up examinations revealed improved clinical status in ten patients (55.6%), stable neurological status in seven (38.9%), and deterioration in one patient (5.5%). At the last visit, 17 out of 18 patients were alive. Our results indicate that stereotactic radiosurgery is an effective and safe treatment modality in the management of glomus jugulare tumors, particularly for residual or previously untreated small tumors.

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Glioma vascularity correlates with reduced patient survival and increased malignancy

Surgical Neurology Volume 72, Issue 3, September 2009, Pages 242-246. doi:10.1016/j.surneu.2008.11.012

The objective of this study was 2-fold: (1) document the presence and degree of vascularity in gliomas of different pathologic grades and (2) determine whether the presence of abnormal vascularity, determined by catheter angiography, correlates with a shortened survival.

Methods As part of a protocol for radiographic data acquisition that was used in a computer-assisted, stereotactic system, all patients who underwent biopsy or resection of a newly diagnosed glioma between 1994 and 2000 at our institution routinely underwent preoperative catheter angiography. The presence and degree of tumor vascularity were recorded and then correlated with survival and pathologic grade. The confounding effects of age, KPS, adjuvant treatment, and extent of resection on survival were considered.

Results Two hundred thirty-one patients were included in this study. The mean follow-up of survivors was 7.8 years. Tumor vascularity correlated with a shortened survival (proportional hazards RR for survival, 0.69; 95% CI, 0.58-0.82). This correlation persisted after correction for age, KPS score, adjuvant therapy, and extent of resection (RR, 0.81; 95% CI, 0.68-0.97). Abnormal vascularity was present in 25 (30%) of 82 low-grade (WHO grade 2) gliomas. Overall, the extent of vascularity (none [120 patients, 52%], blush [63 patients, 27%], neovessels [25 patients, 11%], and arteriovenous shunting [23 patients, 10%]) correlated with worse WHO tumor grade (P < .0001).

Conclusions The presence of abnormal vascularity correlates with both a shortened survival and higher grade of malignancy. These findings underscore the importance of antiangiogenesis factor investigation and drug development for the treatment of gliomas, regardless of their pathologic grade.

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