Impact of tumor characteristics and pre- and postoperative hormone levels on hormonal remission following endoscopic transsphenoidal surgery in patients with acromegaly.

OBJECTIVE: Acromegaly is a disease of acral enlargement and elevated serum levels of insulin-like growth factor-1 (IGF-1) and growth hormone (GH), usually caused by a pituitary adenoma. A lack of consensus on factors that reliably predict outcomes in acromegalic patients following endoscopic endonasal transsphenoidal surgery (EETS) warrants additional investigation. METHODS: The authors identified 52 patients with acromegaly who underwent an endoscopic endonasal approach (EEA) for resection of a GH-secreting pituitary adenoma. Preoperative and postoperative tumor and endocrinological characteristics such as tumor size, invasiveness, and GH/IGF-1 levels were evaluated as potential indicators of postoperative hormonal remission. Endocrinological remission was defined as postoperative IGF-1 levels at or below the age- and sex-normalized values. RESULTS: The 52 patients had a mean age of 50.7 ± 13.4 years and a mean follow-up duration of 24.4 ± 19.1 months. Ten patients (19%) had microadenomas and 42 (81%) had macroadenomas. Five patients (9.6%) had giant adenomas. Forty-four tumors (85%) had extrasellar extension, with 40 (77%) exhibiting infrasellar invasion, 18 (35%) extending above the sella, and 7 (13%) invading the cavernous sinuses. Thirty-six patients (69%) underwent gross-total resection (GTR; mean maximal tumor diameter 1.47 cm), and 16 (31%) underwent subtotal resection (STR; mean maximal tumor diameter 2.74 cm). Invasive tumors were significantly larger, and Knosp scores were negatively correlated with GTR. Thirty-eight patients (73%) achieved hormonal remission after EEA resection alone, which increased to 87% with adjunctive medical therapy. Ninety percent of patients with microadenomas and 86% of patients with macroadenomas achieved hormonal remission. Preoperative IGF-1 and postoperative day 1 (POD1) GH levels were inversely correlated with hormonal remission. Postoperative CSF leakage occurred in 2 patients (4%), and none experienced vision loss, death, or injury to internal carotid arteries or cranial nerves. CONCLUSIONS: Endoscopic transsphenoidal resection of GH-secreting pituitary adenomas is a safe and highly effective treatment for achieving hormonal remission and tumor control in up to 87% of patients with acromegaly when combined with postoperative medical therapy. Patients with lower preoperative IGF-1 and POD1 GH levels, with less invasive pituitary adenomas, and who undergo GTR are more likely to achieve postoperative biochemical remission.

Sporadic and Von-Hippel Lindau disease-associated spinal hemangioblastomas: institutional experience on their similarities and differences.

INTRODUCTION: Hemangioblastomas are uncommon tumors of the central nervous system that can be seen in Von Hippel-Lindau (VHL) disease. Despite their benign histology, hemangioblastomas can cause substantial morbidity due to involvement of critical structures. In order to better understand the clinical behavior of spinal cord hemangioblastomas, we have analyzed the clinical, pathologic, radiologic characteristics and management of sporadic and VHL-associated cases at our institution. METHODS: We performed a database search to identify all spinal hemangioblastomas at our institution between 1997 and 2016. Tumor characteristics were analyzed for sporadic and VHL-associated tumors separately in order to understand the differences in groups. RESULTS: We included 20 patients with VHL-associated spinal hemangioblastomas, and 22 patients with sporadic spinal hemangioblastomas. VHL-associated patients were significantly younger at time of presentation compared to sporadic patients (p < 0.0025). Thirty-two patients (76.2%) presented with focal weakness, 34 (81.0%) with sensory loss, and 22 (52.4%) with pain. VHL patients were more likely to present with multiple symptoms (p < 0.001). Median follow-up time was 20.9 months, during which 17 tumors recurred. The median recurrence free interval was 44 months. There were no differences in gross total resection rates between sporadic and VHL-associated cases (p = 0.197). VHL-associated cases had a higher rate of repeat surgery for recurrence (14 patients-73.6%) compared to sporadic cases (3 patients-13.6%; p < 0.001). CONCLUSION: VHL-associated spinal hemangioblastomas differ from sporadic tumors in terms of age, presenting symptoms, multifocality, and rate of recurrence. Recurrences seem to be unrelated to the extent of resection, indicating the need for life-long follow up for VHL patients.

Approach to the postoperative patient with Cushing's disease.

INTRODUCTION: Cushing's disease is a neuroendocrine disorder marked by hypercortisolemia secondary to overproduction of ACTH by a corticotropic pituitary adenoma. Due to the diverse and deleterious effects of hypercortisolemia including diabetes mellitus, hypertension, and cardiovascular disease, prompt and accurate diagnosis followed by surgical resection of the responsible corticotropic adenoma is critical. METHODS: In the following review, we present a focused synopsis of recently published data and management strategies for the post-operative Cushing's disease patient with a particular focus on studies examining perioperative complications, establishment of biochemical remission, factors associated with disease remission, and predictors of recurrence. CONCLUSIONS: Although no standard definition of remission exists, we suggest measurement of serum cortisol level on the morning of postoperative day 1 given the preponderance of evidence in the published literature suggesting its association with long-term remission and relatively low rates of recurrence. Nevertheless, all patients should be counseled that recurrence can occur in a delayed fashion and that annual endocrine testing should be utilized to track and confirm disease status.

Update on the management of recurrent Cushing's disease.

After transsphenoidal surgery, Cushing's disease (CD) shows excellent long-term remission rates, but it may recur and pose a therapeutic challenge. Findings in recent published reports on the treatment of recurrent adrenocorticotropic hormone (ACTH)-secreting tumors suggest that repeat resection, radiation-based therapies such as Gamma Knife surgery and proton-beam radiosurgery, pharmacotherapy, and bilateral adrenalectomy all have important roles in the treatment of recurrent CD. Each of these interventions has inherent risks and benefits that should be presented to the patient during counseling on retreatment options. Radiation-based therapies increasingly appear to have efficacies similar to those of repeat resection in achieving biochemical remission and tumor control. In addition, an expanding retinue of medication-based therapies, several of which are currently being evaluated in clinical trials, has shown some promise as tertiary adjunctive therapies. Lastly, bilateral adrenalectomy may offer durable control of refractory recurrent CD. An increasing number of published studies with long-term patient outcomes highlight the evolving treatment patterns in the management of recurrent CD.

Altering biomolecular condensates as a potential mechanism that mediates cannabidiol effect on glioblastoma.

Glioblastoma (GBM) is an extremely aggressive primary brain tumor with poor prognosis, short survival time post-diagnosis and high recurrence. Currently, no cure for GBM exists. The identification of an effective therapeutic modality for GBM remains a high priority amongst medical professionals and researches. In recent studies, inhalant cannabidiol (CBD) has demonstrated promise in effectively inhibiting GBM tumor growth. However, exactly how CBD treatment affects the physiology of these tumor cells remains unclear. Stress granules (SG) (a sub-class of biomolecular condensates (BMC)) are dynamic, membrane-less intracellular microstructures which contain proteins and nucleic acids. The formation and signaling of SGs and BMCs plays a significant role in regulating malignancies. This study investigates whether inhaled CBD may play an intervening role towards SGs in GBM tumor cells. Integrated bioinformatics approaches were preformed to gain further insights. This includes use of Immunohistochemistry and flow cytometry to measure SGs, as well as expression and phosphorylation of eukaryotic initiation factor-2α (eIF2α). The findings of this study reveal that CBD receptors (and co-regulated genes) have the potential to play an important biological role in the formation of BMCs within GBM. In this experiment, CBD treatment significantly increased the volume of TIAR-1. This increase directly correlated with elevation in both eIF2α expression and p-eIF2α in CBD treated tissues in comparison to the placebo group (p < 0.05). These results suggest that inhalant CBD significantly up-regulated SGs in GBM, and thus support a theory of targeting BMCs as a potential therapeutic substrate for treating GBM.

Effects of temporal lobectomy on consciousness-impairing and consciousness-sparing seizures in children.

PURPOSE: Most children with medically refractory temporal lobe epilepsy (TLE) become seizure free after temporal lobectomy, but some individuals continue to seize. As studies of temporal lobectomy typically focus on seizure freedom, the effect of surgery on seizure type and frequency among children with persistent seizures is poorly understood. Seizures which impair consciousness are associated with increased morbidity compared to consciousness-sparing seizures. METHODS: A retrospective cohort study was performed to evaluate the effects of temporal lobectomy on seizure type and frequency in children with intractable TLE. RESULTS: Among 58 pediatric TLE patients with a mean (±SEM) age of 14.0 ± 0.7 years who received temporal lobectomy, 46 (79.3%) individuals achieved an Engel class I seizure outcome, including 38 (65.5%) children who became completely seizure free (Engel IA). Mean follow-up was 2.7 ± 0.4 years. While the number of patients experiencing simple partial seizures (SPSs) (consciousness sparing) decreased by only 23 % after surgery, the number of children having complex partial seizures and generalized tonic-clonic seizures (consciousness impairing) diminished by 87 and 83%, respectively (p < 0.01). SPS was the predominant seizure type in only 11.3% of patients before resection, but in 42.1% of patients with postoperative seizures (p < 0.01). Children with postoperative seizures experienced a 70% reduction in overall seizure frequency compared to baseline (p < 0.05), having consciousness-impairing seizures 94% less frequently (p < 0.05), but having consciousness-sparing seizures 35% more frequently (p = 0.73). CONCLUSIONS: Seizure type and frequency are important considerations in the medical and surgical treatment of children with epilepsy, although complete seizure freedom remains the ultimate goal.

Endoscopic-Assisted Microsurgical Resection of a Recurrent Conus Medullaris Dermoid Tumor.

Surgery for intramedullary spinal cord tumors presents multiple unique challenges, including the need to operate through a very narrow myelotomy window. In this operative video, we demonstrate the use of the surgical endoscope in an operation performed on a 35-year-old woman. She has a history of conus medullaris dermoid tumor resection, and she presented to us with worsening lower extremity sensory and motor symptoms. Magnetic resonance imaging showed a 4-cm recurrence of her tumor. After consenting to surgery, she underwent reoperative posterior lumbar approach to resection of her tumor. In our video we demonstrate our use of a surgical endoscope, which allowed us to minimize the extent of our myelotomy and resect tumor rostral and caudal to our myelotomy, thus minimizing the risk of damage to normal spinal cord that is displaced by tumor (Video 1).

The long-term postsurgical prognosis of patients with pineoblastoma.

BACKGROUND: For this report, the authors comprehensively summarized the existing literature on patients with pineoblastoma and identified the variables and treatments that had an impact patient on outcomes. METHODS: A comprehensive search identified 109 studies that collectively described the outcomes of patients with pineoblastoma. Individual patient data were classified based on treatment and were subjected to univariate comparisons. Cox regression analysis included comparisons of survival outcomes controlling for age, extent of resection, and treatment group, and between-group survival comparisons were performed using the Kendall tau (rank correlation) statistic. RESULTS: Two hundred ninety-nine patients met inclusion criteria. The overall survival rate was 54% (175 of 299 patients) at a mean follow-up of 31 ± 1.9 months (range, 1-159 months). The analyses demonstrated a markedly worse prognosis for children aged ≤ 5 years compared with older patients (5-year survival rate: 15% for children aged ≤ 5 years vs 57% for children aged ≥ 5 years; log-rank P < .00001). In addition, a graded increase in survival was observed with increasing degrees of resection (5-year survival rate: 84% for patients who underwent gross total resection vs 53% for patients who underwent subtotal resection vs 29% for patients who underwent debulking; log-rank P < .0001). Multivariate analysis indicated that not achieving gross total resection markedly worsened patient survival (subtotal resection: hazard ratio, 6.47; 95% confidence interval, 2.3-19; P = .001. debulking: hazard ratio, 9.27; 95% confidence interval, 3.2-27; P < .0001). CONCLUSIONS: The current findings emphasize the importance of aggressive surgical resection in the treatment of pineoblastoma. In addition, the authors conclude that clinical trials should not mix young patients with older patients or patients who undergo subtotal resection with patients who undergo gross total resection, because such heterogeneity may alter the variability of responses to treatment and reduce the likelihood of success.

Intracranial hemangiopericytoma: clinical experience and treatment considerations in a modern series of 40 adult patients.

INTRODUCTION: Intracranial hemangiopericytoma (HPC) is a malignant meningothelial tumor. Because of its rarity, few guidelines exist for optimal management. METHODS: University of California at San Francisco patients managed for intracranial HPC were compiled into a single database based on a retrospective review of patient records. Univariate and multivariate regression was performed to determine factors that independently predicted treatment outcomes. RESULTS: A total of 40 patients with intracranial HPC were treated from 1989 to 2010. Treatment and follow-up information was available for analysis on 35 patients. The median survival for all patients was 16.2 years after date of diagnosis, with 1-year, 5-year, and 10-year survival rates of 100%, 92%, and 68%, respectively. Nineteen patients (54%) had HPC recurrence. The median time until recurrence was 5 years, with 1-year, 5-year, and 10-year progression-free survival rates of 96%, 49%, and 28%, respectively. Seven patients (20%) developed extracranial metastasis. Tumor characteristics associated with earlier recurrence included size ≥6 cm (log-rank, P < .05) and nonskull base location (log-rank, P < .05). Strategies combining adjuvant radiation with tumor resection appeared to hinder tumor progression, but had no effect on overall survival or the development of metastasis. Greater extent of resection was associated with increased overall survival (log-rank, P < .05). CONCLUSIONS: Adjuvant radiation may show promise in preventing tumor progression, but recurrence remains a common treatment outcome regardless of initial strategy. When safe and feasible, gross total resection should be pursued as an initial surgical strategy to maximize overall survival. The propensity of these tumors to metastasize makes detailed staging imaging necessary.

Pretemporal Transcavernous Approach for Resection of an Orbito-Cavernous Oculomotor Schwannoma.

The following operative video demonstrates surgical tenets and nuances of the pretemporal transcavernous approach in an unusual case of a 33-year-old patient suffering new onset diplopia and a third nerve palsy due to an orbito-cavernous oculomotor schwannoma. Near total resection was accomplished through an extended pterional craniotomy with pretemporal transcavernous exposure of her lesion, resulting in resolution of her preoperative oculomotor palsy and visual dysfunction. When combined with extended pterional and modified frontotemporal orbitozygomatic approaches, the pretemporal transcavernous approach provides excellent surgical access to the parasellar region including the superior orbital fissure and cavernous sinus. Meticulous dissection and early identification of tissue planes, including cranial nerve and vascular anatomy, allows for safe removal of tumors arising in this region. The link to the video can be found at: https://youtu.be/EuIRTP7wWBQ .

Endoscope-assisted far lateral craniotomy for resection of posterior fossa neurocysticercosis: illustrative case.

BACKGROUND: Neurocysticercosis is a parasitic infection that commonly affects the ventricles, subarachnoid spaces, and spinal cord of the central nervous system. The authors report an unusual manifestation of purely posterior fossa neurocysticercosis treated with endoscope-assisted open craniotomy for resection. OBSERVATIONS: A 67-year-old male presented with 2 months of progressive dizziness, gait ataxia, headaches, decreased hearing, and memory impairment. Imaging revealed an extra-axial cystic lesion occupying the foramen magnum and left cerebellopontine angle with significant mass effect and evidence of early hydrocephalus. Gross-total resection was accomplished via a left far lateral craniotomy with open endoscopic assistance, and pathological findings were consistent with neurocysticercosis. Postoperatively, he was noted to have a sixth nerve palsy, and adjuvant therapy included albendazole. By 9 months postoperatively, he exhibited complete resolution of an immediate postoperative sixth nerve palsy in addition to all preoperative symptoms. His hydrocephalus resolved and did not require permanent cerebrospinal fluid (CSF) diversion. LESSONS: When combined with traditional skull base approaches, open endoscopic techniques allow for enhanced visualization and resection of complex lesions otherwise inaccessible under the microscope alone. Recognition and obliteration of central nervous system neurocysticercosis can facilitate excellent neurological recovery without the need for CSF diversion.

Treatment strategies for giant pituitary adenomas in the era of endoscopic transsphenoidal surgery: a multicenter series.

OBJECTIVE: Given the anatomical complexity and frequently invasive growth of giant pituitary adenomas (GPAs), individually tailored approaches are required. The aim of this study was to assess the treatment strategies and outcomes in a large multicenter series of GPAs in the era of endoscopic transsphenoidal surgery (ETS). METHODS: This was a retrospective case-control series of 64 patients with GPAs treated at two tertiary care centers by surgeons with experience in ETS. GPAs were defined by a maximum diameter of ≥ 4 cm and a volume of ≥ 10 cm3 on preoperative isovoxel contrast-enhanced MRI. RESULTS: The primary operation was ETS in all cases. Overall gross-total resection rates were 64% in round GPAs, 46% in dumbbell-shaped GPAs, and 8% in multilobular GPAs (p < 0.001). Postoperative outcomes were further stratified into two groups based on extent of resection: group A (gross-total resection or partial resection with intracavernous remnant; 21/64, 33%) and group B (partial resection with intracranial remnant; 43/64, 67%). Growth patterns of GPAs were mostly round (11/14, 79%) in group A and multilobular (33/37, 89%) in group B. In group A, no patients required a second operation, and 2/21 (9%) were treated with adjuvant radiosurgery. In group B, early transcranial reoperation was required in 6/43 (14%) cases due to hemorrhagic transformation of remnants. For the remaining group B patients with remnants, 5/43 (12%) underwent transcranial surgery and 12/43 (28%) underwent delayed second ETS. There were no deaths in this series. Severe complications included stroke (6%), meningitis (6%), hydrocephalus requiring shunting (6%), and loss or distinct worsening of vision (3%). At follow-up (mean 3 years, range 0.5-16 years), stable disease was achieved in 91% of cases. CONCLUSIONS: ETS as a primary treatment modality to relieve mass effect in GPAs and extent of resection are dependent on GPA morphology. The pattern of residual pituitary adenoma guides further treatment strategies, including early transcranial reoperation, delayed endoscopic transsphenoidal/transcranial reoperation, and adjuvant radiosurgery.

Epidermal growth factor module-containing mucin-like receptor 2 is a newly identified adhesion G protein-coupled receptor associated with poor overall survival and an invasive phenotype in glioblastoma.

Epidermal growth factor (EGF) module-containing mucin-like receptor 2 (EMR2) is a member of the seven span transmembrane (TM7) adhesion G-protein coupled receptor subclass. It is abundantly expressed in immune cells of myeloid origin and appears to mediate cellular adhesion, migration, and signaling. Based on an analysis showing earlier mortality among glioblastoma patients whose tumors highly express EMR2, we studied its expression patterns in glioblastoma and potential to mediate cellular proliferation and invasion. We performed univariate analysis of overall survival in GBM patients expressing low, moderate, and high amounts of EMR2 based on publicly available microarray data in the Cancer Genome Atlas (TCGA). Using RT-PCR and western blotting, we studied mRNA and protein expression patterns of EMR2, respectively. We then employed siRNA knockdown of EMR2 in two human glioblastoma cell lines expressing high levels of EMR2 to assess functional effects on cellular proliferation and invasion, in vitro. Kaplan-Meier analysis of TCGA survival data for GBM demonstrated that EMR2 levels are inversely correlated with overall time until mortality (log rank, P < 0.01). EMR2 mRNA and protein is variably expressed in primary glioblastoma samples and human glioblastoma cell lines. When comparing cells transfected with siRNA targeting EMR2 to those transfected with a negative, scramble control, there was no difference in proliferation over 72 h in the SF767 and G55 glioblastoma cell lines. However, EMR2 knockout cells demonstrated an almost 3-fold reduction in migration compared to their negative controls (P < 0.05) in the SF767 cell line, and an almost 2-fold reduction (P < 0.05) in migration in the G55 cell line. We provide evidence that EMR2 is expressed in glioblastoma and has significant functional consequences on cellular invasion, but not proliferation. In light of its ability to promote adhesion and migration in immune cells, our data suggest that EMR2 may mediate similar phenomena in glioblastoma. The invasive phenotype conferred by EMR2 correlates with clinical data demonstrating poor survival in glioblastoma patients who express high levels of EMR2 in their tumor.

Factors associated with preservation of facial nerve function after surgical resection of vestibular schwannoma.

Avoidance of facial nerve palsy is one of the major goals of vestibular schwannoma (VS) microsurgery. In this study, we examined the significance of previously implicated prognostic factors (age, tumor size, the extent of resection and the surgical approach) on post-operative facial nerve function. We selected all VS patients from prospectively collected database (1984-2009) who underwent microsurgical resection as their initial treatment for histopathologically confirmed VS. The effect of variables such as surgical approach, tumor size, patient age and extent of resection on rates facial nerve dysfunction after surgery, were analyzed using multivariate logistic regression. Patients with preoperative facial nerve dysfunction (House-Brackman [HB] score 3 or higher) were excluded, and HB grade of 1 or 2 at the last follow-up visit was defined as "facial nerve preservation." A total of 624 VS patients were included in this study. Multivariate logistic regression analysis found that only pre-operative tumor size significantly predicted poorer facial nerve outcome for patients followed-up for ≥6 and ≥12 months (OR 1.27, 95% CI 1.09-1.49, p < 0.01; OR 1.35, 95% CI 1.10-1.67, P < 0.01, respectively). We found no significant relationship between facial nerve function and age, extent of resection, surgical approach, or tumor size (when extent of resection and surgical approach were included in the regression analysis). Because facial nerve palsy is a debilitating and psychologically devastating condition for the patient, we suggest altering surgical aggressiveness in patients with unfavorable tumor anatomy, particularly in cases with large tumors where overaggressive resection might subject the patient to unwarranted risk. Residual disease can be followed and controlled with radiosurgery if interval growth is noted.

Complement and the central nervous system: emerging roles in development, protection and regeneration.

As expanding research reveals the novel ability of complement proteins to promote proliferation and regeneration of tissues throughout the body, the concept of the complement cascade as an innate immune effector has changed rapidly. In particular, its interactions with the central nervous system have provided a wealth of information regarding the ability of complement proteins to mediate neurogenesis, synaptogenesis, cell migration, neuroprotection, proliferation and regeneration. At numerous phases of the neuronal and glial cell cycle, complement proteins exert direct or indirect influence over their behavior and fate. Neuronal stem cells differentiate and migrate in response to complement, and it prevents injury and death in adult cells in response to toxic agents. Furthermore, complement proteins promote survival via anti-apoptotic actions, and can facilitate clearance and regeneration of injured tissues in various models of CNS disease. In summary, we highlight the protean abilities of complement proteins in the central nervous system, underscoring an exciting avenue of research that has yielded greater understanding of complement's role in central nervous system health and disease.

Factors influencing overall survival rates for patients with pineocytoma.

Given its rarity, appropriate treatment for pineocytoma remains variable. As the literature primarily contains case reports or studies involving a small series of patients, prognostic factors following treatment of pineocytoma remain unclear. We therefore compiled a systematic review of the literature concerning post-treatment outcomes for pineocytoma to better determine factors associated with overall survival among patients with pineocytoma. We performed a comprehensive search of the published English language literature to identify studies containing outcome data for patients undergoing treatment for pineocytoma. Kaplan-Meier analysis was utilized to determine overall survival rates. Our systematic review identified 168 total patients reported in 64 articles. Among these patients, 21% underwent biopsy, 38% underwent subtotal resection, 42% underwent gross total resection, and 29% underwent radiation therapy, either as mono- or adjuvant therapy. The 1 and 5 year overall survival rates for patients receiving gross total resection versus subtotal resection plus radiotherapy were 91 versus 88%, and 84 versus 17%, respectively. When compared to subtotal resection alone, subtotal resection plus radiation therapy did not offer a significant improvement in overall survival. Gross total resection is the most appropriate treatment for pineocytoma. The potential benefit of conventional radiotherapy for the treatment of these lesions is unproven, and little evidence supports its use at present.

The complement cascade as a mediator of tissue growth and regeneration.

Recent evidence has demonstrated that the complement cascade is involved in a variety of physiologic and pathophysiologic processes in addition to its role as an immune effector. Research in a variety of organ systems has shown that complement proteins are direct participants in maintenance of cellular turnover, healing, proliferation and regeneration. As a physiologic housekeeper, complement proteins maintain tissue integrity in the absence of inflammation by disposing of cellular debris and waste, a process critical to the prevention of autoimmune disease. Developmentally, complement proteins influence pathways including hematopoietic stem cell engraftment, bone growth, and angiogenesis. They also provide a potent stimulus for cellular proliferation including regeneration of the limb and eye in animal models, and liver proliferation following injury. Here, we describe the complement cascade as a mediator of tissue growth and regeneration.

Craniopharyngioma: a comparison of tumor control with various treatment strategies.

OBJECT: Craniopharyngiomas have a propensity to recur after resection, potentially causing death through their aggressive local behavior in their critical site of origin. Recent data suggest that subtotal resection (STR) followed by adjuvant radiotherapy (XRT) may be an appealing substitute for gross-total resection (GTR), providing similar rates of tumor control without the morbidity associated with aggressive resection. Here, the authors summarize the published literature regarding rates of tumor control with various treatment modalities for craniopharyngiomas. METHODS: The authors performed a comprehensive search of the English language literature to identify studies publishing outcome data on patients undergoing surgery for craniopharyngioma. Rates of progression-free survival (PFS) and overall survival (OS) were determined through Kaplan-Meier analysis. RESULTS: There were 442 patients who underwent tumor resection. Among these patients, GTR was achieved in 256 cases (58%), STR in 101 cases (23%), and STR+XRT in 85 cases (19%). The 2- and 5-year PFS rates for the GTR group versus the STR+XRT group were 88 versus 91%, and 67 versus 69%, respectively. The 5- and 10-year OS rates for the GTR group versus the STR+XRT group were 98 versus 99%, and 98 versus 95%, respectively. There was no significant difference in PFS (log-rank test) or OS with GTR (log-rank test). CONCLUSIONS: Given the relative rarity of craniopharyngioma, this study provides estimates of outcome for a variety of treatment combinations, as not all treatments are an option for all patients with these tumors.

Preservation of facial nerve function after resection of vestibular schwannoma.

OBJECTIVE: Most data regarding facial nerve function in patients undergoing microsurgical resection of vestibular schwannomas predominantly include series performed at a single institution. In an effort to minimise individual surgeon or institutional bias, we performed an analysis of the published literature on facial nerve outcomes following microsurgical resection of vestibular schwannomas. The objective of this study was to provide a comprehensive assessment of reported outcomes for facial nerve preservation after VS surgery. MATERIALS AND METHODS: We identified a total of 296 studies involving over 25,000 patients that included outcome data for facial nerve function of vestibular schwannoma patients treated surgically. Data regarding surgical approach, tumour size, patient age, and use of intra-operative monitoring were extracted and correlated with facial nerve function after surgery. Patients with preoperative facial nerve dysfunction (House-Brackmann score 3 or higher) were excluded and 'facial nerve preservation' was defined as grade I or II House-Brackmann function at last follow-up visit. RESULTS: A total of 79 articles reporting on 11,873 patients met our inclusion criteria contributing to our analysis. Patients treated with the middle cranial fossa approach had a trend towards higher overall facial nerve preservation rate (85%), compared to the translabyrinthine approach (81%, p = 0.07) , and did statistically better than the retrosigmoid approach (78%, p < 0.0001). Patients with an average tumour size <20 mm had significantly improved facial nerve preservation rates, compared to larger tumours (90% vs. 67%, p < 0.0001). Patients under 65 years of age had a lower facial nerve preservation rate (71% vs. 84%, p < 0.001). Finally, the use of intra-operative monitoring improved the facial nerve preservation rate (76% vs. 71%, p < 0.001). CONCLUSION: Factors that appear to be associated with facial nerve preservation after microsurgical resection of a vestibular schwannoma include tumour size <20 mm, use of the middle fossa approach and use of neuromonitoring during surgery. These data provide a summary assessment of the published literature regarding facial nerve preservation after microsurgical resection of vestibular schwannoma.

Transblepharo-Preseptal Modified Orbitozygomatic Approach for Resection of Giant Frontal Cavernous Malformation: 3-Dimensional Operative Video.

A previously healthy 44-year-old woman presented with a first-time seizure. Magnetic resonance imaging (MRI) revealed a right frontal intraaxial mass extending from the orbitofrontal gyri and gyrus rectus to the head of the caudate (Video 1). The mass demonstrated heterogeneous signal intensity on precontrast T1-weighted MRI, minimal contrast enhancement, and mixed intensity on gradient echo MRI sequence consistent with a likely cavernous malformation. Given the location above the orbital roof with cranial-caudal extension to the level of the caudate, a transblepharo-preseptal modified orbitozygomatic craniotomy was recommended. With the assistance of plastic surgery, the lesion was approached through an eyelid incision that extended laterally to expose the keyhole. A McCarty burr hole was made, followed by a tailored orbitozygomatic craniotomy with osteotomies extending through the superolateral orbit and greater sphenoid wing to expose the proximal sylvian fissure. Dura was opened in a C-shaped fashion over the periorbital fat to allow for mild downward retraction of the globe, exposing the subfrontal trajectory. The opticocarotid cistern was opened to allow for cerebrospinal fluid egress and relaxation, and the lesion was readily identified through the use of stereotactic neuronavigation and presence of a faint hemosiderin blush within the underlying parenchyma. The standard microsurgical technique was used to perform a gross total resection of the pathologically confirmed cavernous malformation. The orbitozygomatic bone flap was replaced and plated, and the wound was closed in multiple layers. The patient was seen at a 3-month follow-up without further seizures.