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Objective: The purpose of this article was to determine the impact of employing a telephone clinic for follow-up of patients with stable lateral skull-base tumours., Method: An analysis of 1515 patients in the national lateral skull-base service was performed, and 148 patients enrolled in the telephone clinic to date were identified. The length of time that patients waited for results of their follow-up scans and the travel distance saved by patients not having to attend the hospital for their results was determined., Results: The mean time from scan to receiving results was 30.5 ± 32 days, 14 days sooner than in the face-to-face group (p = 0.0016). The average round-trip distance travelled by patients to the hospital for results of their scans was 256 ± 131 km., Conclusion: The telephone clinic led to a significant reduction in time until patients received their scan results and helped reduce travel distance and clinic numbers in traditional face-to-face clinics.

Introduction: Chondrosarcomas are extremely rare, locally invasive, and potentially mortal malignant cartilaginous tumors. In this study, we aimed to evaluate the incidence and survival rates and trends of skull base chondrosarcomas (SBC)., Methods: Data from SBC patients between 1975 and 2017 were extracted from the Surveillance, Epidemiology, and End Results (SEER) database. The age-adjusted incidence rates (AAR) were calculated for the overall cases and based on gender, age, race, and histology. Furthermore, the relative survival rates for one, three, and five years, and the rates stratified to the aforementioned selected variables were computed. Besides, we conducted a joint point regression analysis to calculate the annual percent change (APC) and its associated standard error (SE) for AAR and mortality., Results: The AAR rate of SBC was 0.019 per 100,000. Higher AAR rates were observed in patients who were in the 65-74-year-age-group, females, Caucasians, and had none mesenchymal subtype. The relative one-year, three-year and five-year-survival rates were 99.58 %, 93.67 %, and 89.10 %, respectively. Lower survival rates were noted in patients who were males, African Americans, and had a mesenchymal subtype. The trend analysis has shown a significant yearly increase (P < 0.001) in AAR of SBC (APC ± SE = 0.0005 %±0.0001), along with a significant yearly decline in mortality rates (APC ± SE= -0.0202 %±0.0029)., Conclusion: Despite the increase in AAR over time, there has been a significant decline in mortality rates over time, which might have been due to the advancement of treatment modalities, improvement in diagnostic imaging, and modification in disease grading., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Objective: NF2 patients can develop new meningiomas throughout their lifetime. Little is known about the clinical features of newly developed NF2 meningiomas. In this study, we analyzed newly developed NF2 meningiomas in a large patient population., Patients and Methods: Among 452 NF2 patients, the location patterns of 81 pediatric and 939 adult NF2 meningiomas were compared to find the predominant locations of newly developed meningiomas in adulthood. The clinical features of 39 newly developed meningiomas in 24 NF2 patients were summarized. Clinical risk factors of NF2 meningioma growth rates were analyzed., Results: Pediatric patients had significantly more intracranial meningiomas than adult patients at the skull base (except for the petrosal region) (p < 0.0063). Adult patients had significantly more cranial meningiomas than pediatric patients at the parasagittal, parafalcine (middle & posterior), and frontal/parietal/cerebellar convex surfaces (p < 0.0063). Newly developed NF2 meningiomas in adults tended to occur at different locations than the locations of NF2 meningiomas in pediatric patients. New meningiomas could develop at various ages. Ninety-five NF2 patients were imaged and followed up for at least one year. Twenty-four patients (25.3 %) developed 39 new meningiomas during the follow-up period. They usually had initial meningiomas when new meningiomas occurred. The number of newly developed meningiomas per patient and the petrosal location were significantly associated with both the absolute and relative annual growth rates (p < 0.05)., Conclusions: The number of newly developed NF2 meningiomas seems to be a clinical marker of NF2 disease severity. In adults, new NF2 meningiomas tend to occur in patients with initial meningiomas. The predominant locations of newly developed NF2 meningiomas seem to be the parasagittal, parafalcine (middle/posterior), and frontal/parietal/cerebellar convex surfaces., Competing Interests: Declaration of Competing Interest None., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Introduction: The incidence of meningiomas among the elderly is considered to be high, and are at increased risk of severe morbidity and mortality following surgery due to their aging physiology and unexpected comorbidities. This study aimed to evaluate the optimal management strategies of meningiomas in elderly patients., Methods: We retrospectively analyzed 150 patients with incidental large (≥ 3 cm) and giant (≥ 6 cm) anterior skull base meningiomas from 2009 to 2018. These patients were divided into elderly group (≥ 65 years, n = 70) and younger group (< 65 years, n = 80). Information of patients with regard to their medical records, operative details, relevant imaging, and follow-up data were obtained from their respective electronic medical records., Results: The elderly patients had significantly longer length of hospital stay (15.9 ± 3.5) compared to younger patients (13.6 ± 3.6, P < 0.001). Karnofsky Performance Scale (KPS) at discharge was significantly lower in elderly group when compared to younger group (P = 0.04). However, the KPS at 1-year after surgery was similar between the two groups. In addition, there was no significant difference in the incidence of surgical complications between the two groups. Multivariate regression analysis of postoperative complications revealed blood loss ≥ 800 mL (P = 0.007) and BMI (< 18.5 or ≥ 24, P < 0.001) as risk factors, rather than age., Conclusions: Surgical resection in elderly patients with incidental anterior skull base large and giant meningiomas is considered to be a safe and effective therapeutic option owing to acceptable mortality, postoperative complications and postoperative clinical outcomes.

Objective: The influence of graft type (nonautologous vs. autologous) on surgical outcomes in endoscopic anterior skull base (EASB) reconstruction is not well understood. This review systematically evaluated rates of postoperative complications of EASB repairs that utilized autologous or nonautologous grafts., Methods: Original studies reporting EASB reconstruction outcomes were extracted from PubMed, Ovid, and the Cochrane Library from database inception to 2019. Risk ratios, risk differences, χ 2 tests, and multivariate logistic regression were used to evaluate outcome measures: postoperative cerebrospinal fluid (CSF) leaks, meningitis, and other major complications (OMCs)., Results: A total of 2275 patients from 29 studies were analyzed. Rates of postoperative CSF leaks, meningitis, and OMCs were 4.0%, 1.6%, and 2.3%, respectively, using autologous grafts, and 5.0%, 0.3%, and 1.0%, respectively, using nonautologous grafts. Multivariate analysis of 118 patients demonstrated no significant differences in age, CSF flow rate, single or multilayer reconstruction, and presence of intraoperative CSF leak or lumbar drain. Meta-analyses of 6 studies yielded a risk ratio of 0.64 (95% confidence interval [CI], 0.19-2.14; P = 0.47) for postoperative CSF leakage, and risk differences of -0.01 (95% CI, -0.06 to 0.05; P = 0.80) and -0.02 (95% CI, -0.09 to 0.05; P = 0.51) for postoperative meningitis and OMCs, respectively. There were no significant differences in postoperative CSF leakage (P = 0.95) and OMCs (P = 0.41) between graft types among cases with intraoperative CSF leaks. However, meningitis rates were lower (P = 0.04) in the nonautologous group., Conclusions: EASB reconstructions utilizing autologous and nonautologous grafts are associated with similar rates of postoperative CSF leakage and OMCs. In cases with intraoperative CSF leakage, nonautologous grafts were associated with reduced postoperative meningitis., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Objective: To gain insight into the role of germline genetics in the development of chordoma, the authors evaluated data from 2 sets of patients with familial chordoma, those with and without a germline duplication of the T gene (T-dup+ vs T-dup-), which was previously identified as a susceptibility mechanism in some families. The authors then compared the patients with familial tumors to patients with sporadic chordoma in the US general population reported to the National Cancer Institute's Surveillance, Epidemiology, and End Results (SEER) program through 2015., Methods: Evaluation of family members included review of personal and family medical history, physical and neurological examination, and pre- and postcontrast MRI of the skull base and spine. Sixteen patients from 6 white families with chordoma had a chordoma diagnosis at family referral. Screening MR images of 35 relatives revealed clival lesions in 6, 4 of which were excised and confirmed to be chordoma. Thus, data were available for 20 patients with histologically confirmed familial chordoma. There were 1759 patients with histologically confirmed chordoma in SEER whose race was known., Results: The median age at chordoma diagnosis differed across the groups: it was lowest in T-dup+ familial patients (26.8 years, range 5.3-68.4 years); intermediate in T-dup- patients (46.2 years, range 11.8-60.1 years); and highest in SEER patients (57 years, range 0-98 years). There was a marked preponderance of skull base tumors in patients with familial chordoma (93% in T-dup+ and 83% in T-dup-) versus 38% in the SEER program (37% in white, 53% in black, and 48.5% in Asian/Pacific Islander/American Indian/Alaska Native patients). Furthermore, 29% of white and 16%-17% of nonwhite SEER patients had mobile-spine chordoma, versus no patients in the familial group. Several T-dup+ familial chordoma patients had putative second/multiple primary chordomas., Conclusions: The occurrence of young age at diagnosis, skull base presentation, or multiple primary chordomas should encourage careful review of family history for patients diagnosed with chordoma as well as screening of at-risk family members by MRI for early detection of chordoma. Furthermore, given genetic predisposition in some patients with familial chordoma, identification of a specific mutation in a family will permit surveillance to be limited to mutation carriers-and consideration should be given for imaging the entire neuraxis in any chordoma patient presenting at an early age or with a blood relative with chordoma. Finally, future studies should explore racial differences in age at diagnosis and presenting site in chordoma.

Squamous cell carcinoma (SCC) is one of the most common malignant neoplasms involving the anterior skull base, resulting from local invasion from an adjacent anatomical region. The primary sites of origin are most frequently the nasal cavity and paranasal sinuses, and less often the skin and orbit. Treatment strategies for these tumors have evolved, although management remains challenging due to the proximity to critical structures and their location at the intracranial-extracranial interface. The rarity of these tumors, and limited numbers at any one institution, has meant that most published series have grouped together different histologies in reporting outcomes. Data on SCC alone are thus limited. Treatment of SCC involving the anterior skull involves a multidisciplinary team approach. A number of potential treatment options exist, dependent on tumor, patient, and institutional factors. Current treatment strategies commonly involve multimodality therapy using a combination of surgery, radiotherapy, and chemotherapy. Primary surgery is considered the mainstay of initial treatment in those tumors which are deemed resectable, followed by adjuvant radiotherapy with or without chemotherapy. Preoperative assessment includes confirmation of the tissue diagnosis, and imaging to assess resectability, guide the surgical approach, and to plan the extent of surgery and method of reconstruction. Analysis of our institutional database for patients undergoing surgery with SCC involving the anterior skull base showed a 5-year disease-specific survival of 65% for sinonasal SCC and 71% for cutaneous SCC. The surgical margin status as well as dural and pterygopalatine fossa involvement were predictors of a poor prognosis., (© 2020 S. Karger AG, Basel.)

Background: Maffucci syndrome (MS) and Ollier disease (OD) are rare diseases characterized by multiple benign enchondromas. The incidence of skull base (SB) enchondromas and the risk of malignant transformation remain unknown. Most SB lesions are asymptomatic, and surgical resection carries significant morbidity. Observation may be a reasonable strategy. We report our experience with observation of probable SB enchondromas in MS/OD patients., Methods: Retrospective review of OD/MS patients with cranial imaging between 1995 and 2018 at 1 institution., Results: 14 patients were included: 3 with MS (21.4%) and 11 with OD (78.6%). The median age was 28 years (range, 11-74 years) and 57.1% were female. Extracranial chondrosarcoma was reported in 3 (21.4%) patients. Seven (50%) patients with SB enchondroma or chondrosarcoma were identified on initial imaging. In patients with SB lesions, the indications for imaging were headache (n=3), seizure (n=1), and diplopia (n=1); 2 cases were incidental findings. The most commonly involved structures were petroclival fissure (86%) and clivus (71%). Treatment included observation (6/7) and resection (1/7). Follow-up imaging was available for all SB lesions, with a mean interval of 50.7 months (range, 5-225 months) and was negative for progression in all patients., Conclusions: Primary SB lesions in OD/MS patients frequently present in the petroclival junction. Cranial screening and close observation should be considered in MS/OD patients, given the increased risk of intra-axial intracranial tumors, de novo chondrosarcomas, or malignant degeneration of previously known lesions. In asymptomatic patients, observation appears to be a safe strategy in this cohort. Further case accumulation and follow-up are required to better understand the long-term outcomes., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Objectives: To investigate the association between certain intracranial masses (meningioma and arachnoid cyst) and the incidence of Pneumosinus Dilatans (PSD) - including whether the size of the mass correlates with severity of the condition., Patients and Methods: A review of the available case reports on PSD was performed. Clinical data was extracted from 111 case reports for analysis. A further case-control study was performed using CT Head datasets to investigate the aetiological relationship between intracranial masses and PSD. Cases included patients with confirmed arachnoid cyst or meningioma. Controls included patients with no intracranial masses., Results: PSD is most common in the frontal (48%) and sphenoid sinuses (43%). Men are twice as likely to be affected as women. 58% of cases occur in patients aged 35 or under. The most common symptoms reported are facial deformities (39%), headache (24%) and visual loss (15%). Unexplained visual changes (e.g. diplopia, reduced visual acuity) are strongly correlated with sphenoid sinus involvement. PSD is more common in patients with skull-base meningioma (OR 5.67) and middle cranial fossa arachnoid cysts (OR 10.00). Mean sinus volume in patients with PSD can increase by up to 4 times., Conclusion: We present the first direct investigation into the relationship between meningioma, arachnoid cyst and Pneumosinus Dilatans. There is a statistical correlation between skull-base meningioma and middle cranial fossa arachnoid cysts and the incidence of PSD. This specific anatomical relation suggests that local factors contribute to the pathogenesis of the condition. Alterations in intracranial pressure due to mass effect or vascular occlusion, in addition to the localised release of bone growth factors (IGF-1, IGF-2, PDGF), are possible mechanisms for this. The first peak in incidence of PSD coincides with the completion of normal sinus pneumatisation, which raises the further possibility that predisposing genetic factors also contribute., (Copyright © 2019 Elsevier B.V. All rights reserved.)

Purpose: We aim to delineate the clinical characteristics of patients with primary intracranial solitary plasmacytoma (PISPC) and prognostic factors for their outcomes., Methods: This study retrospectively reviewed 17 patients with PISPC from our center and an additional 70 cases of PISPC published previously to analyze outcome predictors., Results: The entire cohort included 38 (43.7%) males and 49 (56.3%) females with a mean age of 54 years. Skull base tumors were found in 49 (56.3%) patients. Gross total resection (GTR) was achieved in 31 (35.6%) patients. Postoperative adjuvant treatments, including radiotherapy (RT) alone, chemotherapy (CMT) alone, and RT + CMT were administered in 49 (56.3%) patients, 3 (3.5%) patients, and 16 (18.4%) patients, respectively. After a median follow-up of 24 (mean 42.4) months, the 5-year disease progression-free survival (PFS), recurrence-free survival (RFS), multiple myeloma (MM)-free survival (MMFS), and overall survival (OS) were 52.9%, 76.2%, 69.6%, and 76.1%, respectively. Multivariate analysis unveiled that a skull base tumor location (HR 2.395, p = 0.040) and no RT (HR 3.115, p = 0.004) were negative prognostic factors for PFS, no RT (HR 10.526, p = 0.003) for RFS, each 1-year increase in age (HR 1.039, p = 0.049) for MMFS, and increasing age (HR 1.052, p = 0.043) and CMT (HR 6.022, p = 0.005) were risk factors for OS. However, GTR did not benefit the aforementioned outcomes., Conclusion: For patients with presumed PISPC, a biopsy followed by RT is recommended for skull base PISPC. However, the role of CMT is still not clear. Our findings need to be verified in a larger prospective cohort in the future. Systematic review registration number CRD42018098782.

Background and Aims: The aim of this systematic review is to describe the epidemiology of chordoma and to provide a clear overview of clinical prognostic factors predicting progression-free and overall survival., Methods: Four databases of medical literature were searched. Separate searches were performed for each of the two objectives. Reference and citation tracking was performed. Papers were processed by two independent reviewers according to a protocol that included risk of bias analysis. Disagreement was resolved by discussion. Pooled analyses were planned if homogeneity of data would allow., Results: Incidence-incidence rates ranged between 0.18 and 0.84 per million persons per year and varied between countries and presumably between races. On average patients were diagnosed in their late fifties and gender data indicate clear male predominance. Two of the largest studies (n = 400 and n = 544) reported different anatomical distributions: one reporting the skull base and sacrococcygeal area affected in 32% and 29% of cases, whereas the other reporting that they were affected in 26% and 45% of cases, respectively., Prognostic Factors: Statistically significant adverse prognostic factors predicting progression-free and overall survival include female sex, older age, bigger tumour size, increasing extent of tumour invasion, non-total resection, presence of metastasis, local recurrence, and dedifferentiated histological subtype., Conclusions: Incidence rate and anatomical distribution vary between countries and presumably between races. Most chordomas arise in the skull base and sacrococcygeal spine, and the tumour shows clear male predominance. Multiple adverse prognostic factors predicting progression-free and overall survival were identified in subgroups of patients. These slides can be retrieved under Electronic Supplementary Material.

Objective: Arrested pneumatization of the sphenoid sinus is a developmental anatomic variation but may be confused with serious diseases of the skull base. The purpose of this study was to investigate the prevalence of arrested pneumatization of the sphenoid sinus in paediatric patients with haemotologic diseases like sickle cell anaemia, thalassemia and leukemia. Materials and Methods: One hundred and eight paediatric patients (43 girls, 65 boys; age range: 4-18 years; median age: 13 years) with haemotologic diseases who underwent at least one magnetic resonance imaging of the head and neck between 2010 and 2017 in a single institution were included. Magnetic resonance imaging studies were retrospectively reviewed. Well-defined and fat-containing non-expansile lesions located at the sphenoid sinus region were diagnosed as arrested pneumatization of the sphenoid sinus. Medical diagnoses, lesion sizes, age and sex of the patients were recorded. Results: Five (4.6%) of 108 patients demonstrated arrested pneumatization of the sphenoid sinus (3 girls, 2 boys; age range: 9-18 years; median age: 14 years). One (1.2%) of 83 patients with acute leukemia, 2 (15%) of 13 patients with sickle cell anaemia and 2 of 12 (17%) patients with thalassemia demonstrated arrested pneumatization of the sphenoid sinus. No statistical correlation was found between arrested pneumatization of the sphenoid sinus and possible risk factors like patient age and sex. Conclusions: Patients with haemoglobinopathies like sickle cell anaemia and thalassemia involving red-blood-cells had higher prevalence of arrested pneumatization of the sphenoid sinus than patients with leukemia which involves the white-blood-cells. This supported the suggestion that regional blood-flow disorders induce arrested pneumatization of the sphenoid sinus. Correct diagnosis prevents invasive procedures like biopsy and surgery.

Objective: Iatrogenic tumor seeding after open surgery for chordoma has been well described in the literature. The incidence and particularities related to endoscopic endonasal surgery (EES) have not been defined., Methods: The authors retrospectively reviewed their experience with EES for clival chordoma, focusing on cases with iatrogenic seeding. The clinical, radiographic, pathological, and molecular characterization data were reviewed., Results: Among 173 EESs performed for clival chordomas at the authors' institution between April 2003 and May 2016, 2 cases complicated by iatrogenic seeding (incidence 1.15%) were identified. The first case was a 10-year-old boy, who presented 21 months after an EES for a multiply recurrent clival chordoma with a recurrence along the left inferior turbinate, distinct from a right petrous apex recurrence. Both appeared as a T2-hypertintense, T1-isointense, and heterogeneously enhancing lesion on MRI. Resection of the inferior turbinate recurrence and debulking of the petrous recurrence were both performed via a purely endoscopic endonasal approach. Unfortunately, the child died 2 years later due progression of disease at the primary site, but with no sign of progression at the seeded site. The second patient was a 79-year-old man with an MRI-incompatible pacemaker who presented 19 months after EES for his clival chordoma with a mass involving the floor of the left nasal cavity that was causing an oro-antral fistula. On CT imaging, this appeared as a homogeneously contrast-enhancing mass eroding the hard palate inferiorly, the nasal septum superiorly, and the nasal process of the maxilla, with extension into the subcutaneous tissue. This was also treated endoscopically (combined transnasal-transoral approach) with resection of the mass, and repair of the fistula by using a palatal and left lateral wall rotational flap. Adjuvant hypofractionated stereotactic CyberKnife radiotherapy was administered using 35 Gy in 5 fractions. No recurrence was appreciated endoscopically or on imaging at the patient's last follow-up, 12 months after this last procedure. In both cases, pathological investigation of the original tumors revealed a fairly aggressive biology with 1p36 deletions, and high Ki-67 levels (10%-15%, and > 20%, respectively). The procedures were performed by a team of right-handed surgeons (otolaryngology and neurosurgery), using a 4-handed technique (in which the endoscope and suction are typically passed through the right nostril, and other instruments are passed through the left nostril without visualization)., Conclusions: Although uncommon, iatrogenic seeding occurs during EES for clival chordomas, probably because of decreased visualization during tumor removal combined with mucosal trauma and exposure of subepithelial elements (either inadvertently or because of mucosal flaps). In addition, tumors with more aggressive biology (1p36 deletions, elevated Ki-67, or both) are probably at a higher risk and require increased vigilance on surveillance imaging and endoscopy. Further prospective studies are warranted to evaluate the authors' proposed strategies for decreasing the incidence of iatrogenic seeding after EES for chordomas.

Background: Skull base chordomas in children are extremely rare. Their course, management, and outcome have not been defined., Objective: To describe the preeminent clinical and radiological features in a series of pediatric patients with skull base chordomas and analyze the outcome of a cohort who underwent uniform treatment. We emphasize predictors of overall survival and progression-free survival, which aligns with Collins' law for embryonal tumors., Methods: Thirty-one patients with a mean age of 10.7 yr (range 0.8-22) harboring skull base chordomas were evaluated. We retrospectively analyzed the outcomes and prognostic factors for 18 patients treated by the senior author, with uniform management of surgery with the aim of gross total resection and adjuvant proton-beam radiotherapy. Mean follow-up was 119.2 mo (range 8-263)., Results: Abducens nerve palsy was the most common presenting symptom. Imaging disclosed large tumors that often involve multiple anatomical compartments. Patients undergoing gross total resection had significantly increased progression-free survival (P = .02) and overall survival (P = .05) compared with those having subtotal resection. Those who lived through the period of risk for recurrence without disease progression had a higher probability of living entirely free of progression (P = .03; odds ratio = 16.0). Age, sex, and histopathological variant did not yield statistical significance in survival., Conclusion: Long-term overall and progression-free survival in children harboring skull base chordomas can be achieved with gross surgical resection and proton-beam radiotherapy, despite an advanced stage at presentation. Collins' law does apply to pediatric skull base chordomas, and children with this disease have a high hope for cure.

Background: The aim of this retrospective study was to investigate the long-term seizure control and antiepileptic drug (AED) prescriptions, as well as identifying predictors of seizure(s) before and after surgery in a population-based cohort of operated intracranial meningioma patients., Methods: A total of 113 consecutive adult (> 18 years old) patients with newly diagnosed meningioma operated at the Karolinska University Hospital between 2006 and 2008 were included and followed up until the end of 2015. Data on seizure activity and AED prescriptions were obtained through chart review and telephone interview. Logistic regression and survival analysis were applied to identify risk factors for pre- and postoperative seizures., Results: A total of 21/113 (18.6%) patients experienced seizures before surgery of which 8/21 (38.1%) went on to become seizure-free after surgery. Thirteen (14%) patients experienced new-onset seizures after surgery. The regression analysis revealed tumor diameter ≥ 3.5 cm as a risk factor for preoperative seizures (OR 3.83, 95% CI 1.14-12.87). Presence of headache (OR 0.19, 95% CI 0.05-0.76) and skull base tumor location (OR 0.14, 95% CI 0.04-0.44) decreased the risk of preoperative seizures. Postoperative seizures were associated with tumor diameter ≥ 3.5 cm (OR 2.65, 95% CI 1.06-6.62) and history of preoperative seizures (OR 3.50, 95% CI 1.55-7.90)., Conclusion: Seizures are common before and after intracranial meningioma surgery. Approximately one third of patients with preoperative seizures become seizure-free on long-term follow-up after surgery, while 14% experienced new-onset seizures after surgery. Larger tumor size, absence of headache, and non-skull base location were associated with preoperative seizures, while tumor size and preoperative seizures were associated with postoperative seizures.

Metastatic disease to the intracranial dura, the calvarium, and the skull base is relatively uncommon but presents unique diagnostic and management challenges in the patient with cancer. Modern imaging techniques have facilitated the detection of intracranial tumor deposits, leading to increased incidence. While dural and calvarial metastases often present with nonspecific symptoms, skull base metastases present with distinct clinical syndromes dependent on the local neurovascular structures affected. Intracranial dural metastases can often be confused with meningioma and pose a diagnostic challenge, as well as significant neurologic morbidity, especially in the setting of hemorrhage. Surgical intervention may be helpful in selected patients for symptomatic relief as well as survival benefit. Management paradigms need to take into account the relative risks, benefits, and likely outcomes for each possible modality of treatment. Surgical excision is useful in many patients and in combination with radiation therapy can provide significant palliation. While medical therapy is rarely an initial therapy in these entities, it may be of added benefit dependent on the underlying tumor histology and prior treatment history. Occasionally treatment with curative intent is justified., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Background: Limited data exist to guide the multimodality management of chondrosarcomas (CSAs) arising in the skull base., Objective: To determine the impact of histological subtype/grade on progression-free survival (PFS) and the indications for surgery, radiation, and chemotherapy based on histology., Methods: A retrospective review was performed of 37 patients (conventional type: 81%, mesenchymal: 16.2%, dedifferentiated: 2.7%) treated at The University of Texas M.D. Anderson Cancer Center. Of the conventional subtype, 23% were grade 1, 63% were grade 2, and 14% were grade 3. In addition to surgery, mesenchymal/dedifferentiated CSAs (18% of the cohort) underwent neoadjuvant chemotherapy and 48.6% of the overall cohort received adjuvant radiotherapy. Histological grade/subtype and treatment factors were assessed for impact on median PFS (primary outcome)., Results: Conventional subtype vs mesenchymal/dedifferentiated was positively associated with median PFS (166 vs 24 months, P < .05). Increasing conventional grade inversely correlated with median PFS ( P < .05). Gross total resection positively impacted PFS in conventional CSAs (111.8 vs 42.9 months, P = .201) and mesenchymal/dedifferentiated CSAs (58.2 vs 1.0 month, P < .05). Adjuvant radiotherapy significantly impacted PFS in conventional grades 2 and 3 (182 vs 79 months, P < .05) and a positive trend with mesenchymal/dedifferentiated CSAs (43.5 vs 22.0 months). Chemotherapy improved PFS for mesenchymal/dedifferentiated CSAs (50 vs 9 months, P = .089)., Conclusion: There is a potential need for histological subtype/grade specific treatment protocols. For conventional CSAs, surgery alone provides optimal results grade 1 CSAs, while resection with adjuvant radiotherapy yields the best outcome for grade 2 and 3 CSAs. Improvements in PFS seen with neoadjuvant therapy in mesenchymal/dedifferentiated CSAs indicate a potential role for systemic therapies. Larger studies are necessary to confirm the proposed treatment protocols., (Copyright © 2017 by the Congress of Neurological Surgeons)

Primary lymphomas of the skull base are exceedingly rare and thus not commonly dealt with in everyday clinical practice. Primary diffuse large B cell lymphoma is the most commonly found (30-40% of primary non-Hodgkin lymphomas of the bone). This article reports a case of primary lymphoma of the skull base and reviews all the relevant literature in the PubMed, National Institutes of Health Library, and Google Scholar databases to outline the clinical, diagnostic, and surgical traits of this yet widely unexplored pathology. The incidence of cases peaks between 60 and 70 years of age; those affected tend to be mostly male. The clinical presentation of this pathology is usually abrupt with headache and acute deficit of cranial nerves that improves dramatically with intravenous corticosteroid therapy. The abducens nerve is most commonly involved. Imaging can be unclear because corticosteroid medications can significantly alter magnetic resonance imaging findings, at least in the early stages of the disease, similarly to what happens for primary brain lymphomas ("the ghost tumor"). Cavernous sinus, parasellar region, upper clivus, and Meckel cave are usually found to be already affected by the time a diagnosis can be made. The intracavernous internal carotid artery is usually encased by the lesion rather than displaced. Because of the anatomical pattern of primary lymphomas of the skull base and their well-known chemosensitivity, surgery is usually only used for diagnosis. Surgical approaches for primary lymphoma of the skull base include transcranial and transnasal-transsphenoidal endoscopy and microsurgery as well as other minimally invasive techniques. Due to the rarity of this neoplasm, there are no definitive data regarding the overall survival rate among patients., (Georg Thieme Verlag KG Stuttgart · New York.)

Background: Although intraoperative magnetic resonance imaging (iMRI) increasingly is used during glioma resection, its role in skull base surgery has not been well documented. In this study, we evaluate our experience with iMRI for skull base surgery., Methods: Medical records were reviewed retrospectively on all neurosurgical cases performed at our institution in the IMRIS iMRI suite between April 2014 and July 2015., Results: During the study period, the iMRI suite was used for 71 skull base tumors. iMRI was performed in 23 of 71 cases. Additional tumor resection was pursued after scanning in 7 of 23 patients. There was a significant difference in procedure length between the scanned versus nonscanned groups, and this was likely attributable to a greater proportion of petroclival meningiomas in the scanned group. Further analyses revealed significant increases in procedure length for the following scanned subgroups: anterolateral approach, anterolateral and petroclival lesion locations, and meningiomas. The rate of non-neurologic complications was significantly greater in the scanned group, particularly for patients with tumors >3 cm., Conclusions: Despite the unique challenges associated with skull base tumor surgery, iMRI can be safely obtained while adding a modest although not prohibitive amount of time to the procedure. The immediate evidence of residual tumor with a patient still in position to have additional resection may influence the surgeon to alter the surgical plan and attempt further resection in a critical area., (Copyright © 2016 Elsevier Inc. All rights reserved.)

OBJECT Paragangliomas are highly vascular head and neck tumors for which preoperative embolization is often considered to facilitate resection. The authors evaluated their initial experience using a dual-lumen balloon to facilitate preoperative embolization in 5 consecutive patients who underwent preoperative transarterial Onyx embolization assisted by the Scepter dual-lumen balloon catheter between 2012 and 2014. OBJECT The authors reviewed the demographic and clinical records of 5 patients who underwent Scepter-assisted Onyx embolization of a paraganglioma followed by resection between 2012 and 2014. Descriptive statistics of clinical outcomes were assessed. RESULTS Five patients (4 with a jugular and 1 with a vagal paraganglioma) were identified. Three paragangliomas were embolized in a single session, and each of the other 2 were completed in 3 staged sessions. The mean volume of Onyx used was 14.3 ml (range 6-30 ml). Twenty-seven vessels were selectively catheterized for embolization. All patients required selective embolization via multiple vessels. Two patients required sacrifice of parent vessels (1 petrocavernous internal carotid artery and 1 vertebral artery) after successful balloon test occlusion. One patient underwent embolization with Onyx-18 alone, 2 with Onyx-34 alone, and 1 with Onyx-18 and -34. In each case, migration of Onyx was achieved within the tumor parenchyma. The mean time between embolization and resection was 3.8 days (range 1-8 days). Gross-total resection was achieved in 3 (60%) patients, and the other 2 patients had minimal residual tumor. The mean estimated blood loss during the resections was 556 ml (range 200-850 ml). The mean postoperative hematocrit level change was -17.3%. Two patients required blood transfusions. One patient, who underwent extensive tumor penetration with Onyx, developed a temporary partial cranial nerve VII palsy that resolved to House-Brackmann Grade I (out of VI) at the 6-month follow-up. One patient experienced improvement in existing facial nerve weakness after embolization. CONCLUSIONS Scepter catheter-based Onyx embolization seems to be safe and effective. It was associated with excellent distal tumor vasculature penetration and holds promise as an adjunct to conventional transarterial Onyx embolization of paragangliomas. However, the ease of tumor penetration should encourage caution in practitioners who may be able to effect comparable improvement in blood loss with more conservative proximal Onyx penetration.

Background: Traditionally, the surgical removal of tuberculum sellae meningioma (TSM) and olfactory groove meningioma (OGM) requires transcranial approaches and microsurgical techniques, but in the last decade endoscopic expanded endonasal approaches have been introduced: transcribriform for OGMs and transtuberculum-transplanum for TSM. A comparative analysis of the literature concerning the two types of surgical treatment of OGMs and TSM is, however, difficult., Methods: We conducted a literature search using the PubMed database to compare data for endoscopic and microsurgical techniques in the literature. We also conducted a retrospective analysis of selected cases from our series presenting favorable characteristics for an endoscopic approach, based on the criteria of operability of these lesions as generally accepted in the literature, and we compared the results obtained in these patients with those in the endoscopic literature., Results: We believe that making the sample more homogeneous, the difference between microsurgical technique and endoscopic technique is no longer so striking. A greater radical removal rate, a reduced incidence of cerebrospinal fluid fistula and, especially, the possibility of removing lesions of any size are advantages of transcranial surgery; a higher percentage of improvement in visual outcome and a lower risk of a worsening of a pre-existing deficit or onset of a new deficit are advantages of the endoscopic technique., Conclusion: At present, the microsurgical technique is still the gold standard for the removal of the anterior cranial fossa meningiomas of all sizes, and the endoscopic technique remains a second option in certain cases., (Copyright © 2016 Elsevier Inc. All rights reserved.)

OBJECT The aim of this study was to evaluate the value of intraoperative neurophysiological monitoring (IONM) using electromyography (EMG), brainstem auditory evoked potentials (BAEPs), and somatosensory evoked potentials (SSEPs) to predict and/or prevent postoperative neurological deficits in pediatric patients undergoing endoscopic endonasal surgery (EES) for skull base tumors. METHODS All consecutive pediatric patients with skull base tumors who underwent EES with at least 1 modality of IONM (BAEP, SSEP, and/or EMG) at our institution between 1999 and 2013 were retrospectively reviewed. Staged procedures and repeat procedures were identified and analyzed separately. To evaluate the diagnostic accuracy of significant free-run EMG activity, the prevalence of cranial nerve (CN) deficits and the sensitivity, specificity, and positive and negative predictive values were calculated. RESULTS A total of 129 patients underwent 159 procedures; 6 patients had a total of 9 CN deficits. The incidences of CN deficits based on the total number of nerves monitored in the groups with and without significant free-run EMG activity were 9% and 1.5%, respectively. The incidences of CN deficits in the groups with 1 staged and more than 1 staged EES were 1.5% and 29%, respectively. The sensitivity, specificity, and negative predictive values (with 95% confidence intervals) of significant EMG to detect CN deficits in repeat procedures were 0.55 (0.22-0.84), 0.86 (0.79-0.9), and 0.97 (0.92-0.99), respectively. Two patients had significant changes in their BAEPs that were reversible with an increase in mean arterial pressure. CONCLUSIONS IONM can be applied effectively and reliably during EES in children. EMG monitoring is specific for detecting CN deficits and can be an effective guide for dissecting these procedures. Triggered EMG should be elicited intraoperatively to check the integrity of the CNs during and after tumor resection. Given the anatomical complexity of pediatric EES and the unique challenges encountered, multimodal IONM can be a valuable adjunct to these procedures.

A review of literature presents up-to-date information on the prevalence, incidence, clinical manifestations, diagnosis, and classification of malignant primary skull base tumors (MPSBTs). In the structure of total cancer incidence, malignant head and neck tumors account for 5% of all annual cancer deaths in the USA and are among the 5 most common groups of tumors in males worldwide. These tumors develop most often in the sixth decade of life, occurring 2 times more often in males than in females. In Russia, the MPSBT incidence (as of 2012) was 0.62% out of all newly diagnosed malignant tumors. The incidence rate amounts to 0.66 per 100 000 population and is significantly higher than the global rate (0.44 per 100 000). About half of all malignant skull base tumors have the epithelial nature and affect the anterior parts of the skull base. The most frequent histological types of malignant skull base tumors are squamous cell carcinoma, adenocarcinoma, and non-Hodgkin's B cell lymphoma. Treatment of skull base tumors is an interdisciplinary problem and the area of interest of otolaryngologists, dentists, ophthalmologists, neurosurgeons, plastic surgeons, radiologists, and chemotherapists. Physical and endoscopic examinations, endoscopic transnasal biopsy, CT, MRI, PET/CT, and ultrasound are typically used for verification of the diagnosis, tumor staging, and selection of the treatment approach. The review describes the criteria for TNM staging of malignant tumors of the nasal cavity and paranasal sinuses in accordance with the 7th version of the TNM recommendations of the American Joint Committee on Cancer (AJCC). The TNM-based staging depends on the location (maxillary sinus, nasal cavity, or ethmoid labyrinth) and histological structure of the tumor, which, in turn, determines the tactics of comprehensive treatment and a prognosis group.

The objective of the present work was to describe the poorly known history of the development of the nomenclature of glomus tumours (chemodectomas) of the base of the skull, elucidate etiology and pathogenesis of these neoplasms. The authors present a chronological table illustrating the progress of etiological and pathogenetic studies as well as the surgical treatment of the tumours. The results of analysis and discussion of the controversial issues of the nomenclature are reported.

Recent advances in pediatric cancer treatment have improved disease control and survival outcomes for childhood cancers survivors, including those treated for primary central nervous system and skull base malignancies. Future research in this population will focus on identifying risk factors for infertility, novel screening techniques and recommendations, and quality-of-life outcomes improvement. The purpose of this review is to define the infertility complications observed in pediatric cancer survivors who receive cranial irradiation for central nervous system and skull base malignancies., (Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.)

This study describes the prevalence and distribution of 42 cases of skull base neoplasia in dogs between 2000 and 2014. The average age of affected individuals was 9.5 years, and there was no sex or breed predisposition. The most common skull base neoplasms were meningioma (25 cases) and pituitary adenoma (9 cases). Less common tumors included craniopharyngioma (2 cases), nerve sheath tumor (2 cases), and 1 case each of pituitary carcinoma, meningeal oligodendrogliomatosis, presumed nasal or sinonasal carcinoma, and multilobular tumor of bone. All neoplasms caused some degree of compression of adjacent structures. The distribution of the tumors was greatest in the sellar region (n = 18), followed by the paranasal region (n = 12), caudal cranial fossa (n = 10), central cranial fossa (n = 1), and rostral cranial fossa (n = 1)., (© 2015 The Author(s).)

This study aims to demonstrate survival rates and treatment patterns among patients with chordomas of the skull base using a large population database. Patients with cranial chordomas between 1973 and 2009 were identified from the USA Surveillance, Epidemiology, and End Results (SEER) public use database. Kaplan-Meier analysis was used to examine the effect of surgery and radiation on overall survival. We identified 394 patients with histologically-confirmed cranial chordomas. Median survival was 151 months. Most patients (89.09%) underwent surgery. Less than half (44.92%) received radiation after diagnosis. Patients who underwent surgical resection survived significantly longer than those who did not undergo resection, regardless of other treatments (151 versus 81 months, p<0.001). Ten year survival was lower among patients receiving radiation (44.8% versus 61.4%, p=0.66). Surgery predicted better overall survival by univariate analysis (hazard ratio [HR] 0.603, p=0.0293); younger age at diagnosis (HR 1.028, p<0.001), and later year of diagnosis (HR 0.971, p=0.0027) were prognostic of improved survival in a multivariate model. In subgroup analysis of patients with documented tumor size, smaller tumor size (HR 1.021, p=0.0067), younger age (HR 1.031, p=0.001), and treatment within a higher volume registry (HR 0.490, p=0.0129) predicted improved survival. Surgical intervention offers survival benefit for cranial chordomas. Findings of decreased survival in patients receiving radiation may be associated with selection. Studies examining surgical extent of resection data and radiation details are needed to determine the impact of radiotherapy., (Copyright © 2014 Elsevier Ltd. All rights reserved.)

This study aims to demonstrate survival rates and treatment patterns among patients with chondrosarcomas of the skull base using a large population database. Patients with skull base chondrosarcomas between 1973 and 2009 were identified from the USA Surveillance, Epidemiology, and End Results (SEER) database. Kaplan-Meier survival analysis was used to examine the effect of surgery and radiation on overall survival. We identified 226 patients with skull base chondrosarcomas. Median follow-up was 5.4 years. Median overall survival was 22 years, and 10 year survival was 68.2%. Most patients underwent surgery (92.5%). Few received radiation after diagnosis (38.1%). Ten year survival for all patients treated with surgery was significantly increased compared to those without surgery (69.3% versus 53.9%, p=0.02). There was a significant difference in survival amongst treatment groups (p=0.02), with median overall survival not yet reached for patients who received surgery and radiation (median follow-up 5.3 years), compared to 22 years for non-irradiated surgical patients. Surgery predicted better overall survival by univariate analysis (hazard ratio [HR] 0.420, p=0.03). Female sex (HR 0.470, p=0.011), younger age at diagnosis (HR 1.046, p<0.0001), and later year of diagnosis (HR 0.949, p=0.0006) were prognostic of improved survival in a multivariate model. In subgroup analysis of patients with documented tumor size, smaller tumor size (HR 1.054, p=0.0003) and younger age (HR 1.021, p=0.0067) predicted improved survival. This population based study further reaffirms the role of surgery as an effective treatment for skull base chondrosarcoma as previously reported in small case series. Adjuvant radiation may also confer survival benefit. Optimal treatment strategy has yet to be defined in the literature., (Copyright © 2014 Elsevier Ltd. All rights reserved.)

Object: Chordomas of the skull base are rare and locally invasive and have a poor prognosis. The aim of this retrospective multicenter study was to evaluate the current pattern of care and clinical course and to identify prognostic factors., Methods: A total of 47 patients (26 men; mean age 48.5 years) treated in 5 centers were included. Histology was centrally reviewed; additionally, semiquantitative N- and E-cadherin expression analysis was performed. Prognostic factors were obtained from multivariate regression models. For survival analysis the Kaplan-Meier method was used., Results: The median follow-up period was 5.2 years. Complete resection, incomplete resection, and extended biopsy were performed in 14.9%, 80.9%, and 4.3% of patients, respectively. Surgical morbidity was not associated with extent of resection. Adjuvant radiation therapy was performed in 30 (63.8%) of 47 patients. The median progression-free survival (PFS) was 7.3 years. Complete resection prolonged median overall survival (OS) (p = 0.04). Male patients presented with worse PFS (4.8 years vs 9.8 years; p = 0.04) and OS (8.3 years vs not reached; p = 0.03) even though complete resection was exclusively achieved in the male subpopulation. Multivariate analysis confirmed male sex as the most important risk factor for tumor progression (p = 0.04) and death (p = 0.02). Age, duration of symptoms, initial Karnofsky Performance Scale score, brainstem compression, involvement of the petrous bone, infiltration of the dura mater, modality and dose of radiation therapy, and the E- and N-cadherin expression patterns did not gain prognostic relevance., Conclusions: In skull base chordomas, male patients bear a higher risk of progressive disease and death. Male patients might benefit from more aggressive adjuvant therapy and/or from a closer follow-up schedule.

Background and Purpose: Distinguishing BNCT from chordoma with imaging is critical because of the profound differences in prognosis and management. Yet few reports define the variable imaging characteristics of BNCT. This study aims to evaluate the prevalence and characteristics of BNCT., Methods: A total of 916 patients with 64-section CT and 1.5T MR imaging through the posterior fossa between 2004 and 2009 were evaluated to catalogue the prevalence, clinical presentation, morphology, and imaging properties associated with BNCT., Results: BNCTs were identified in 7 patients (imaging prevalence of 0.76%). All were midline, T1 hypointense, and T2 hyperintense. When present, the bony stalk often associated with EP measured between 1.65 and 3.72 mm. Five cases demonstrated atypical features such as absence of bony stalk (one case), arterial enhancement (one case), clival erosion (four cases), clinical symptoms (one case), and mass effect (one case)., Conclusion: Many notochordal lesions do not fit neatly into the diagnostic criteria for either EP or chordoma. It may be useful to consider these atypical cases along a spectrum of notochord remnant lesions. Close inspection of imaging reveals BNCTs at a similar frequency to its pathologic prevalence. BNCTs such as EP vary in size and may be easily overlooked., (Copyright © 2013 by the American Society of Neuroimaging.)

Background: Injury to the internal carotid artery (ICA) during endoscopic endonasal skull base surgery is a feared complication that is not well studied or reported., Objective: To evaluate the incidence, to identify potential risk factors, and to present management strategies and outcomes of ICA injury during endonasal skull base surgery at our institution., Methods: We performed a retrospective review of all endoscopic endonasal operations performed at our institution between 1998 and 2011 to examine potential factors predisposing to ICA injury. We also documented the perioperative management and outcomes after injury., Results: There were 7 ICA injuries encountered in 2015 endonasal skull base surgeries, giving an incidence of 0.3%. Most injuries (5 of 7) involved the left ICA, and the most common diagnosis was chondroid neoplasm (chordoma, chondrosarcoma; 3 of 7 [2% of 142 cases]). Two injuries occurred during 660 pituitary adenoma resections (0.3%). The paraclival ICA segment was the most commonly injured site (5 of 7), and transclival and transpterygoid approaches had a higher incidence of injury, although neither factor reached statistical significance. Four of 7 injured ICAs were sacrificed either intraoperatively or postoperatively. No patient suffered a stroke or neurological deficit. There were no intraoperative mortalities; 1 patient died postoperatively of cardiac ischemia. One of the 3 preserved ICAs developed a pseudoaneurysm over a mean follow-up period of 5 months that was treated endovascularly., Conclusion: ICA injury during endonasal skull base surgery is an infrequent and manageable complication. Preservation of the vessel remains difficult. Chondroid tumors represent a higher risk and should be resected by surgical teams with significant experience.

Background: Endoscopic skull base surgery is now the preferred treatment option to remove skull base tumors., Objective: To evaluate the patient's sense of smell and mucociliary clearance time (MCT) after skull base surgery., Methods: Patients with pituitary adenoma underwent a transnasal transsphenoidal endoscopic approach (TTEA group, n = 36), whereas patients with other benign parasellar tumors underwent an expanded endonasal approach (EEA group, n = 14) with a vascularized septal flap. Assessment of symptoms (Visual Analogue Scale), olfactometry (Barcelona Smell Test, BAST-24), and MCT (saccharin test) were performed before and 3 months after surgery., Results: Before surgery, patients reported poorer BAST-24 scores on detection, identification, and forced choice than the healthy population, but both study groups had similar sinonasal symptoms, BAST-24, and MCT scores. After surgery, no changes in symptom scores (Visual Analogue Scale) were observed except for the loss of smell (26.7 ± 30.5 mm, P < .05) and posterior nasal discharge (29.7 ± 30.3 mm, P < .05) compared with baseline (5.2 ± 11.3, 19.1 ± 25.3, respectively). EEA patients reported higher loss of smell and posterior nasal discharge compared with TTEA. TTEA and EEA groups had similar scores on postoperative BAST-24. After surgery, however, patients showed prolonged saccharin test (15.6 ± 10.8 min, P < .05) compared with baseline (8.4 ± 4.4 min). In addition, EEA patients reported longer MCT than TTEA patients., Conclusion: EEA but not TTEA has a short-term (3 months) negative impact on patient's olfaction and mucociliary clearance. Patients should be informed about smell loss as a consequence of skull base surgery to prevent legal claims. Likewise, further research and some modifications on reconstruction flaps are encouraged to avoid damaging the olfactory neuroepithelium.

Background: About 90 % of meningiomas are benign (WHO grade I), atypical and anaplastic variants exist (WHO grade II/III, 10 %). Tumour grade has important implications for management. Non-invasive diagnosis of tumour grade is still not feasible. The purpose of this survey was to analyse epidemiological risk factors such as sex, age and location for a higher grade (WHO grade II/III) meningioma in a large surgical series., Methods: A retrospective study comprising 1,663 patients operated on for an intracranial meningioma in a single tertiary-care centre. The population was analysed for correlations including WHO grade, histological subtype, tumour localisation, patient age and gender. Additionally correlations between Ki67 index/WHO grade and localisation were analysed., Results: A binary logistic regression analysis revealed non-skull base localisation (OR 1.779 [CI 1.069-2.960, p = 0.0027]) and age ≥65 years (OR 1.549 [CI 1.214-2.624, p = 0.012]) as significant risk factors for a higher WHO grade. Male gender showed a trend for a higher risk in χ(2) analysis. An analysis of the Ki67 index revealed an increased index for non-skull base localisation compared with skull base (p < 0.001). Correlation analysis of Ki67 distribution in WHO grade I meningiomas revealed higher Ki67 indices for non skull base localisation (p = 0.0024)., Conclusions: Non-skull base localisation and age ≥65 years are independent risk factors for higher grade meningiomas. In other terms, the malignant potential of skull base meningiomas is low. This information is important when advising a patient about individual treatment options (observation, surgery or radio-surgery) and prognosis.

Primary temporal bone tumors are rare. Suspicious lesions of the ear canal should be biopsied for diagnosis. Surgical resection to achieve negative margins is the mainstay of treatment. Small tumors can be treated with lateral temporal bone resection. Parotidectomy and neck dissection are added for disease extension and proper staging. Higher staged tumors generally require subtotal temporal bone resection or total temporal bone resection. Adjuvant postoperative radiotherapy has shown improved survival for some patients. Chemotherapy has an emerging role for advanced stage disease. Evaluation and management by a multidisciplinary team are the best approach for patients with these tumors., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Chondrosarcomas are indolent but invasive chondroid malignancies that can form in the skull base. Standard management of chondrosarcoma involves surgical resection and adjuvant radiation therapy. This review evaluates evidence from the literature to assess the importance of the surgical approach and extent of resection on outcomes for patients with skull base chondrosarcoma. Also evaluated is the ability of the multiple modalities of radiation therapy, such as conventional fractionated radiotherapy, proton beam, and stereotactic radiosurgery, to control tumor growth. Finally, emerging therapies for the treatment of skull-base chondrosarcoma are discussed., (Copyright © 2013. Published by Elsevier Inc.)

Esthesioneuroblastoma is a rare malignant neoplasm in the olfactory region of the nasal cavity and anterior skull base. Diagnosis and staging require anatomic imaging and careful pathologic assessment. Standard treatment is anterior craniofacial resection with postoperative irradiation. The role for chemotherapy is not defined, but is generally for the most advanced cases and used in the neoadjuvant setting and/or postoperatively with irradiation. Prognosis is favorable; however, metastasis rates remain relatively high. Regional and distant metastasis portends a poor outcome. Intensity-modulated radiation treatment and endoscopic surgery have reduced morbidity, but outcomes with these techniques must be fully evaluated., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Background: The advances in endoscopic skull base surgery have led to the resection of increasingly larger cranial base lesions and the creation of larger skull base defects with the potential for increased postoperative high-flow cerebrospinal fluid (CSF) leaks. These concerns led to the development of the vascularized pedicled nasoseptal flap (PNSF), which is now used as the mainstay for repair of large skull base defects in many academic centers. In this report, we review the incidence of postoperative CSF leaks in our institution in patients undergoing endoscopic skull base repair of high-flow CSF leaks with a vascularized PNSF without concurrent CSF diversion., Methods: We performed a retrospective analysis at our tertiary care medical center on patients who underwent endoscopic repair of high-flow CSF leaks using a PNSF without CSF diversion between July 2008 and August 2011. Repair materials, incidence of postoperative CSF leaks, and demographic data were collected., Results: Fifty-nine high-flow CSF leaks were repaired with a PNSF and other repair materials, without the use of lumbar catheter drainage. No postoperative CSF leak occurred in this cohort of patients. The overall postoperative CSF leak rate was 0%., Conclusion: Meticulous multilayer-closure of skull base defects is critical to prevent postoperative CSF leaks. Although lumbar drainage may be useful in select scenarios, it carries inherent risks of intracranial hypotension and pneumocephalus, and may not be necessary for routine management of high-flow CSF leaks in conjunction with a robust PNSF. Further prospective randomized controlled studies may be warranted to evaluate the efficacy of postoperative lumbar drainage., (Copyright © 2012 American Rhinologic Society-American Academy of Otolaryngic Allergy, LLC.)

Background: Postoperative cerebrospinal fluid leak is a significant risk after endoscopic skull base surgery. Recently, novel reconstructive techniques using vascularized pedicled mucosal flaps have been applied to decrease this potential risk. Complete mucosal extirpation in the wound bed is not always feasible and the impact of insetting the flap over intact underlying mucosa is not clear. The purpose of this study was to determine the rate of mucocele formation after nasoseptal flap reconstruction without mucosal stripping., Methods: This is an Institutional Review Board-approved, retrospective study consisting of 28 patients undergoing skull base reconstruction using a pedicled nasoseptal flap between 2008 and 2010 at a tertiary care hospital. In all cases the sinus or skull base mucosa surrounding the defect was left intact. Patients were followed postoperatively by endoscopy and/or imaging for evidence of mucocele formation in the reconstructive bed., Results: The total rate of mucocele formation was 3.6% (1 of 28, noted on postoperative day 46). The mean follow-up time was 243 ± 174 days (range, 46-585 days). Eleven patients were followed for over 1 year. All flaps remained viable and well vascularized., Conclusion: The pedicled nasoseptal flap is an effective means of reconstruction after endoscopic skull base surgery. Avoidance of extensive stripping of the surrounding mucosa does not result in a significant rate of postoperative mucocele formation in the short term. Long-term follow-up is still indicated.

Objective: Primary desmoid-type fibromatoses arising from the oral and maxillofacial region are uncommon and hold the character of local invasion and regional recurrence. The purpose of this study was to analyze the clinicoepidemiologic characteristics of this rare disease in an eastern Chinese population., Study Design: Epidemiological and clinicopathological data on 20 patients were reviewed from the hospital records and analyzed accordingly., Results: Twelve males and 8 females with a ratio of 3:2 were involved. The mandible was the most common site of presentation. Suspected malignant change was found in 6 cases and an extremely high recurrence rate was also found in up to 53%. Abnormally high blood levels of alkaline phosphatase were found in 65% of patients, which had a close relation with tumor malignant change., Conclusion: Chinese people are more susceptible to desmoid-type fibromatoses and are at an extremely high risk of regional malignant change. Elevated serum alkaline phosphatase levels may be taken as an indicator for malignant transformation., (Copyright © 2011 Mosby, Inc. All rights reserved.)

Tumours affecting the nose, paranasal sinuses and adjacent skull base are fortunately rare. However, they pose significant problems of management due their late presentation and juxtaposition to important anatomical structures such eye and brain. The increasing application of endonasal endoscopic techniques to their excision offers potentially similar scales of resection but with reduced morbidity. The present document is intended to be a state-of-the art review for any specialist with an interest in this area 1. to update their knowledge of neoplasia affecting the nose, paranasal sinuses and adjacent skull base; 2. to provide an evidence-based review of the diagnostic methods; 3. to provide an evidence-based review of endoscopic techniques in the context of other available treatments; 4. to propose algorithms for the management of the disease; 5. to propose guidance for outcome measurements for research and encourage prospective collection of data. The importance of a multidisciplinary approach, adherence to oncologic principles with intent to cure and need for long-term follow-up is emphasised.

Purpose of Review: Technological advances and technical innovations have spearheaded an expansion in the indications for endoscopic sinonasal surgery, which now extend to the resection of sinonasal and skull base malignancies. This review examines recent advances that have contributed to make this technique feasible and reviews the basis of adopting an endonasal approach., Recent Findings: Oncologic goals developed for traditional skull base surgery can be safely applied to endoscopic skull base surgery. This has been made possible with the advent of better understanding of skull base anatomy from the endonasal perspective, as well as the development of better instrumentation, better hemostatic materials and more reliable reconstructive techniques. Recent publications have reported similar short-term outcomes for endoscopic skull base resection as compared with traditional approaches with lower complication rates., Summary: Transnasal endoscopic surgery is an important part of the surgical armamentarium for the treatment of sinonasal and skull base malignancies. New technologies will further expand the limits of what can be achieved endoscopically. Collaborative multiinstitutional studies with long-term follow-up are crucial in defining the role of endoscopic resection in the treatment of skull base malignancies.

Background: The aim is to study the clinical, radiological and pathological features of childhood and adolescent meningiomas and analyse outcome prognosticators., Method: A retrospective analysis of the case records of patients less than 20 years of age operated for a meningioma in our institute since 1982 was performed. The variables analysed included age, sex, presentation, associated neurofibromatosis (NF), imaging characteristics, extent of resection and histopathology., Results: The study group included 20 males and 18 females with a mean age of 15.53 years. Eleven children (28.9%) had evidence of NF of whom three had NF2 with bilateral vestibular schwannomas. The common presenting symptoms were seizures (76.3%), raised intracranial tension (71%), and focal neurological deficits (39.4%). The location of the operated tumours were as follows: ten skull base (24.4%), ten falx/parasagittal (24.4%), eight spinal (19.5%), five convexity (12.2%), three posterior fossa (7.3%), three intraventricular (7.3%) and two optic nerve sheath (4.9%). Two children (4.9%) had cystic meningiomas. Grade I excision was achieved only in twenty tumours (48.8%). On histopathology, thirty (73.2%) were grade I, nine (21.9%) were grade II and two (4.9%) were grade III meningiomas. Seven tumours recurred of which six were located at the skull base. During the mean follow up period of 4.74 years, the majority, 32 (84.2%) had a good outcome and five (13.2%) had a poor outcome. One child (2.6%) expired due to post-operative sepsis., Conclusion: Childhood meningiomas are uncommon but not rare lesions with a marginal male predominance. Absence of large series with long follow up precludes any definite conclusions on the clinical course and outcome. Uniform observations made in different series including ours, include a higher incidence of the skull base location and tumours with atypical histopathology. Favourable prognostic factors include younger age (< than 10 years), superficial location, total excision and absence of neurofibromatosis. Location and extent of excision appear to be more important than histopathology grade in predicting outcome.

Aims: (1) Learn if margins of resection in advanced anterior skull base tumors that are resected via endoscopic-assisted means are comparable with traditional craniofacial resection (CFR). (2) Understand the difference in patient morbidity with endoscopic surgery compared to traditional CFR., Methods: Retrospective review of 41 patients undergoing surgery for comparably staged advanced malignancies of the anterior skull base between 2000 and 2006. Eighteen patients underwent endoscopic surgery and 23 patients underwent traditional CFR for American Joint Committee on Cancer stage III, IV or Kadish stage C lesions of the paranasal sinuses and anterior skull base. Margins were evaluated and follow-up ranged from 3 months to 5 years., Results: Of the 18 patients undergoing endoscopic resections, margins were positive/close in 17% (3/18). Margins were positive/close in 17% (4/23) of patients undergoing CFR. Five complications were seen in 4/18 patients (22%) undergoing endoscopic surgery and 11 postoperative complications were seen in 7/23 patients (30%) undergoing CFR. Mean duration of hospitalization was 5.22 and 7.17 days for the endoscopic and CFR groups, respectively., Conclusions: There does not appear to be increased risk of positive margins in patients with advanced lesions undergoing endoscopic-assisted resection. Furthermore, the incidence of morbidity and duration of stay may be improved with endoscopic techniques.

Introduction: Literature specifically focusing on clinoidal meningiomas is scant, particularly with regards to the postoperative visual outcome. In this study, we aimed to document the incidence of optic canal involvement (OCI) by the tumor, its management using a skull base technique, and its significance with relation to the visual outcome., Materials and Methods: Fifty-two patients with clinoidal meningiomas were retrospectively analyzed. In 47 patients, skull base technique consisting of extradural anterior clinoidectomy with falciform ligament and optic nerve sheath opening was performed. Pre-operative visual status and post-operative outcome were analyzed with respect to OCI., Results: The incidences of OCI was present in 19 (36%) and pre-operative visual deficit (VD) in 24 (46%) patients. With regard to pre-operative visual status, OCI was seen in 14 (58%) of 24 patients with VD, as compared to five (18%) in 28 patients without (p = 0.004). Among the 22 patients with VD and detailed postoperative neuro-ophthalmological evaluation, 17 (77%) had visual improvement, and in five patients (23%), vision was unchanged. In the presence of OCI in 11 patients, vision improved in seven (64%), and remained unchanged in four patients (36%), whereas all but one of the 11 patients (91%) without OCI improved and in the remaining one (9%), remained unchanged. Simpson Grade I and II resection was achieved in 71%., Conclusion: OCI is observed in 36% of clinoidal meningiomas, and it correlates well with pre-operative visual status. With the use of the skull base technique, without which the tumor in the optic canal could not have been removed completely and safely, visual improvement of 77% and stability of 23% was achieved.

Skull base, including optic nerve, cavernous sinus, clival and foramen magnum tumors represent a major challenge for neurosurgeons and neuro-oncologists. Growth regulatory signaling pathways for these tumors are of increasing interest as potential targets for new chemotherapy. Those differentially activated in various grades of meningiomas are currently being identified as well. This article reviews some recent findings pathways that appear to regulate meningioma growth. Potential targets for novel therapies are also discussed.

Purpose: To evaluate patients with squamous cell carcinoma of the paranasal sinus and skull base for factors that might predict clinical outcome., Methods: A multi-institutional 13-year retrospective review of anterior skull base malignancies., Results: Of 73 patients with anterior skull base malignancies, squamous cell carcinoma was the most prevalent-30 patients or 41%. Twenty-three patients underwent craniofacial surgery with or without adjuvant chemotherapy. Seven patients, deemed unresectable or not willing to have surgery, were treated with standard radiation protocols often with chemotherapy. The 3- and 5-year survival rates after surgery were 32% and 16%, respectively, compared to a 28% survival rate at 3 and 5 years for the nonsurgical group. Most tumors were in advanced stages accounting for a relatively poor survival in both groups. A Cox regression analysis demonstrated that age (P = .0172) was an independent determinant of poor outcome. Although 3- and 5-year survival of tumors free of sphenoid sinus, dura, retromaxillary, and ptyerygoid space, and orbit treated with surgery showed no significant difference to those patients with involvement, their median time of survival was increased for all anatomical regions., Conclusions: Squamous cell carcinoma of the sinus invading the skull base carries a very poor prognosis regardless of treatment modality. Surgery with adjunctive radiotherapy and/or chemotherapy offers a survival advantage over nonsurgical methods, but treatment should be individualized weighing prognostic factors, such as age, stage, and anatomical extension with morbidity of treatment.

Objective: To report postoperative mortality, complications, and outcomes in a subset of patients with the histologic diagnosis of malignant melanoma extracted from an existing database of a large cohort of patients accumulated from multiple institutions., Design: Retrospective outcome analysis., Setting: Seventeen international tertiary referral centers performing craniofacial surgery for malignant skull base tumors., Patients: A total of 53 patients were identified from a database of 1307 patients who had craniofacial resection for malignant tumors at 17 institutions. The median age was 63 years. Of the 53 patients, 25 (47%) had had prior single modality or combined treatment, which included surgery in 22 (42%), radiation in 11 (21%), and chemotherapy in 2 (4%). The margins of resection were close or microscopically positive in 7 (13%). Adjuvant radiotherapy was given in 22 (42%), chemotherapy in 3 (6%), and vaccine or interferon therapy in 2 (4%). Complications were classified into overall, local, central nervous system, systemic, and orbital. Overall survival (OS), disease-specific survival (DSS), and recurrence-free survival (RFS) were determined using the Kaplan-Meier method. Predictors of outcome were identified by multivariate analysis., Results: Postoperative mortality occurred in 3 patients (6%) and postoperative complications were reported in 14 patients (26%). Local wound complications occurred in 6 patients (11%), central nervous system in 7 (13%), systemic in 3 (6%), and orbital in 1 (2%). With a median follow-up of 10 months (range, 1-159 months), the 3-year OS, DSS, and RFS rates were 28.2%, 29.7%, and 25.5%, respectively. The extent of orbital involvement and adjuvant postoperative radiation therapy (PORT) were independent predictors of DSS and OS on multivariate analysis, whereas only PORT was an independent predictor of RFS. Patients treated with PORT had significantly better 3-year OS (39% vs 18%; relative risk, 2.9; P = .007), DSS (41% vs 19%; relative risk, 3.0; P = .007), and RFS (39% vs 15%; relative risk, 4.2; P = .001)., Conclusions: Craniofacial resection in patients with malignant melanoma of the skull base has mortality (6%) and complication rates (26%) comparable to other malignant tumors of the skull base. However, malignant melanoma is associated with a much poorer OS, DSS, and RFS. Adjuvant PORT correlated with improved 3-year OS, DSS, and RFS on multivariate analysis. These factors must be taken into account when considering craniofacial resection in a patient with malignant melanoma invading the skull base.

Object: The authors analyze the factors that predispose to persistent hydrocephalus in children with posterior fossa tumors and compare their results and treatment policy with those described in the literature, particularly with regard to the higher postoperative shunt insertion rates, which have led some authors to the routine use of preoperative third ventriculostomy., Methods: The clinical records of 180 children treated for posterior fossa tumors in the Department of Pediatric Neurosurgery of the National Institute of Neurosurgery, Budapest, Hungary, between 1990 and 2000 were retrospectively reviewed., Conclusions: The low postoperative shunt insertion rate in our series (15.5%) led us to believe that the routine use of preoperative third ventriculostomy is not entirely justified. Factors such as patient's age and tumor type, which showed a statistically significant association with the postoperative shunt requirement in our study, should be considered when the decision regarding treatment is made.

Object: Historically poor outcomes have been characteristic in patients with lateral skull base malignancies. As advances in skull base surgical techniques have been made, complete resection has increasingly been achieved. This has resulted in improved survival rates and local tumor control., Methods: The authors performed a retrospective review of 95 patients treated for lateral skull base malignancies. The mean age of the patients was 49.4 years. There were 44 females and 51 males. The mean follow-up period was 50 months. Resection was performed in all patients, and postoperative radiotherapy was undertaken in 54% of the cases. Local disease control was maintained in 73% of the patients. Tumor involvement of the facial nerve and intracranial tumor extension did not jeopardize the rate of local control., Conclusions: Despite the fact that technical advances in skull base surgery have resulted in a higher incidence of complete tumor resection and improved survival rates, a respect for the poor prognosis historically associated with lateral skull base malignancies should be maintained and treatment should be appropriately aggressive.