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Skull base chondrosarcoma is a rare tumour usually treated by surgery and proton therapy. However, as mortality rate is very low and treatment complications are frequent, a less aggressive therapeutic strategy could be considered. The objective of this study was to compare the results of surgery only vs surgery and adjuvant proton therapy, in terms of survival and treatment adverse effects, based on a retrospective series. Monocentric retrospective study at a tertiary care centre. All patients treated for a skull base grade I and II chondrosarcoma were included. We collected data concerning surgical and proton therapy treatment and up-to-date follow-up, including Common Terminology Criteria for Adverse Events (CTCAE) scores. 47 patients (23M/24F) were operated on between 2002 and 2015; mean age at diagnosis was 47 years-old (10-85). Petroclival and anterior skull base locations were found in 34 and 13 patients, respectively. Gross total resection was achieved in 17 cases (36%) and partial in 30 (64%). Adjuvant proton therapy (mean total dose 70 GyRBE,1.8 GyRBE/day) was administered in 23 cases. Overall mean follow-up was 91 months (7-182). Of the patients treated by surgery only, 8 (34%) experienced residual tumour progression (mean delay 51 months) and 5 received second-line proton therapy. Adjuvant proton therapy was associated with a significantly lower rate of relapse (11%; p = 0.01). There was no significant difference in 10-year disease specific survival between patients initially treated with or without adjuvant proton therapy (100% vs 89.8%, p = 0.14). Difference in high-grade toxicity was not statistically significant between patients in both groups (25% (7) vs 11% (5), p = 0.10). The most frequent adverse effect of proton therapy was sensorineural hearing loss (39%). Long-term disease specific survival was not significantly lower in patients without adjuvant proton therapy, but they experienced less adverse effects. We believe a surgery only strategy could be discussed, delaying as much as possible proton therapy in cases of relapse. Further prospective studies are needed to validate this more conservative strategy in skull base chondrosarcoma.

PurposeThe long-term use of cyproterone acetate (CPA) is associated with an increased risk of developing intracranial meningiomas. CPA discontinuation most often induces a stabilization or regression of the tumor. The underlying biological mechanisms as well as the reasons why some meningiomas still grow after CPA discontinuation remain unknown. We reported a series of patients presenting CPA-induced meningiomatosis with opposed tumor evolutions following CPA discontinuation, highlighting the underlying histological and genetic features.MethodsPatients presenting several meningiomas with opposite tumor evolution (coexistence of growing and shrinking tumors) following CPA discontinuation were identified. Clinical and radiological data were reviewed. A retrospective volumetric analysis of the meningiomas was performed. All the growing meningiomas were operated. Each operated tumor was characterized by histological and genetic analyses.ResultsFour women with multiple meningiomas and opposite tumor volume evolutions after CPA discontinuation were identified. Histopathological analysis characterized the convexity and tentorial tumors which continued to grow after CPA discontinuation as fibroblastic meningiomas. The decreasing skull base tumor was characterized as a fibroblastic meningioma with increased fibrosis and a widespread collagen formation. The two growing skull base meningiomas were identified as meningothelial and transitional meningiomas. The molecular characterization found two NF2 mutations among the growing meningiomas and a PIK3CA mutation in the skull base tumor which decreased.ConclusionTo our knowledge, this is the first report describing an atypical tumor evolution of CPA-associated meningiomas after CPA discontinuation. The underlying biological mechanisms explaining this observation and especially the close relationship between mutational landscapes and embryologic origins of the meninges in CPA-related meningiomas as well as their clonal origin require further research.

BackgroundSelective amygdalohippocampectomy (AH) is a surgical option for patients with medically intractable seizures from mesial temporal lobe pathology. The transcranial route is considered the best method to achieve this goal. However, the standard approach through the neocortex is still invasive. The risks can be minimized if the mesial temporal lobe is resected while preserving the lateral temporal lobe and the Meyer’s loop. This study explores the feasibility of selective AH by endoscopic endonasal approach (EEA) in cadaveric specimens.MethodsThe endoscopic anatomy of the mesial temporal lobe and the feasibility of a successful selective AH were studied in six hemispheres from three injected human cadavers. Quantitative analyses on the extent of resection and angles of exposure were performed based on CT and MRI studies of pre- and post-selective AH and measurements taken during dissections.ResultsThe EEA V1-V2 corridor provided a direct and logical line of access to the mesial temporal lobe, following its natural trajectory with no brain retraction and minimal exposure of the pterygopalatine fossa. The components of the mesial temporal lobe were resected just as selectively and easily as the transcranial route, but without compromising the structures of the lateral temporal lobe or the Meyer’s loop.ConclusionsThe EEA V1-V2 corridor demonstrated its selective resectability and accessibility of the mesial temporal lobe in cadaveric specimens. The clinical value of this approach should be explored responsibly by a surgeon with both competent microsurgical skills and experiences in EEA.

PurposeSecondary empty sella syndrome (SESS) following pituitary surgery remains a diagnostic and therapeutic challenge. The aim of this study was to specify the diagnostic criteria, surgical indications and results of chiasmapexy in the SESS.MethodsThree cases from two experienced neurosurgical centers were collected and the available literature was reviewed.ResultsThe 3 patients were operated for a giant non-functioning pituitary adenoma, a cystic macroprolactinoma, and an arachnoid cyst respectively. Postoperative visual outcome was initially improved, and then worsened progressively. At the time of SESS diagnosis, visual field defect was severe in all cases with optic nerve (ON) atrophy in 2 cases. Patients were operated via an endoscopic endonasal extradural approach. One patient was re-operated because of early fat reabsorption. Visual outcome improved in 1 case and stabilized in 2 cases. Statistical analyses performed on 24 cases from the literature review highlighted that patient age and severity of the preoperative visual defect were respectively significant and nearly significant prognostic factors for visual outcome, unlike the surgical technique.ConclusionT2-weighted or CISS/FIESTA sequence MRI is mandatory to visualize adhesions, ON kinking and neurovascular conflict. TS approach is the most commonly used approach. The literature review could not conclude on the need for an intra or extradural approach suggesting case by case adapted strategy. Intrasellar packing with non-absorbable material such as bone should be considered. Severity of the visual loss clearly decreases the visual outcome suggesting early chiasmapexy. In case of severe and long standing symptoms before surgery, benefits and surgical risks should be carefully balanced.

AbstractObjectiveTo assess the risk of meningioma associated with use of high dose cyproterone acetate, a progestogen indicated for clinical hyperandrogenism.DesignObservational cohort study.SettingData from SNDS, the French administrative healthcare database, between 2007 and 2015.Participants253 777 girls and women aged 7-70 years living in France who started cyproterone acetate between 2007 and 2014. Participants had at least one reimbursement for high dose cyproterone acetate and no history of meningioma or benign brain tumour, or long term disease status. Participants were considered to be exposed when they had received a cumulative dose of at least 3 g during the first six months (139 222 participants) and very slightly exposed (control group) when they had received a cumulative dose of less than 3 g (114 555 participants). 10 876 transgender participants (male to female) were included in an additional analysis.Main outcome measureSurgery (resection or decompression) or radiotherapy for one or more intracranial meningiomas.ResultsOverall, 69 meningiomas in the exposed group (during 289 544 person years of follow-up) and 20 meningiomas in the control group (during 439 949 person years of follow-up) were treated by surgery or radiotherapy. The incidence of meningioma in the two groups was 23.8 and 4.5 per 100 000 person years, respectively (crude relative risk 5.2, 95% confidence interval 3.2 to 8.6; adjusted hazard ratio 6.6, 95% confidence interval 4.0 to 11.1). The adjusted hazard ratio for a cumulative dose of cyproterone acetate of more than 60 g was 21.7 (10.8 to 43.5). After discontinuation of cyproterone acetate for one year, the risk of meningioma in the exposed group was 1.8-fold higher (1.0 to 3.2) than in the control group. In a complementary analysis, 463 women with meningioma were observed among 123 997 already using cyproterone acetate in 2006 (risk of 383 per 100 000 person years in the group with the highest exposure in terms of cumulative dose). Meningiomas located in the anterior skull base and middle skull base, particularly the medial third of the middle skull base, involving the spheno-orbital region, appeared to be specific to cyproterone acetate. An additional analysis of transgender participants showed a high risk of meningioma (three per 14 460 person years; 20.7 per 100 000 person years).ConclusionsA strong dose-effect relation was observed between use of cyproterone acetate and risk of intracranial meningiomas. A noticeable reduction in risk was observed after discontinuation of treatment.

BackgroundThe type of sellar barrier observed between a pituitary tumor and cerebrospinal fluid (CSF) on preoperative magnetic resonance imaging (MRI) may predict intraoperative CSF leak during endonasal pituitary surgery. This is the first multicentric prospective cohort trial to study the sellar barrier concept and CSF leak rate during endoscopic pituitary surgery.MethodsThis multi-center, international study enrolled patients operated for pituitary adenomas via fully endoscopic endonasal surgery over a period of 4 months. The independent variable was the subtype of sellar barrier observed on preoperative MRI (strong, mixed or weak); the dependent variable was the presence of an intraoperative CSF leak. The primary goal was to determine the association between a particular type of sellar barrier and the risk of intraoperative CSF leak. Appropriate statistical methods were then applied for data analysis.ResultsOver the study period, 310 patients underwent endoscopic endonasal surgery for pituitary tumor. Preoperative imaging revealed a weak sellar barrier in 73 (23.55%), a mixed sellar barrier in 75 (24.19%), and a strong sellar barrier in 162 (52.26%) patients. The overall rate of intraoperative CSF leak among all patients was 69 (22.26%). A strong sellar-type barrier was associated with significantly reduced rate of intraoperative CSF leak (RR = 0.08; 95% CI 0.03–0.19; p < 0.0001), while a weak sellar barrier associated with higher rates of CSF leak (RR = 8.54; 95% CI 5.4–13.5; p < 0.0001).ConclusionsThe preoperative MRI of pituitary patients can suggest intraoperative CSF leak rates, utilizing the concept of the sellar barrier. Patients with a weak sellar barrier carry a higher risk for an intraoperative CSF leak, whereas a strong sellar barrier on MRI seems to mitigate intraoperative CSF leak. We propose that preoperatively assessment of the sellar barrier can prepare surgeons for intraoperative CSF leak repair.

Background Oculomotor nerve palsy (ONP) is a common clinical manifestation of ruptured or unruptured posterior communicating artery (PcomA) aneurysms. Although microsurgical clip ligation has been proven a safe and durable treatment, endovascular management is emerging as an increasingly popular alternative. The aim of our study is to compare the recovery rate from ONP and assess the safety and long-term durability of both techniques. Methods We have reviewed the retrospective data concerning twenty-two patients treated at our institution between 2004 and 2012 for PcomA aneurysms with ONP. Seven patients were operated on via a standard pterional approach, and fourteen were treated by endovascular occlusion with coils. One patient was managed conservatively. Pre-treatment and post-treatment severity of ONP was recorded, as well as the duration of symptoms before admission and treatment-related complications. Results All seven patients who underwent surgery improved, with six total recoveries and one partial recovery. Among the fourteen patients treated by embolization, thirteen initially recovered, but long-term follow-up revealed three cases of exacerbation of ONP after refilling of the aneurysms. Two of them were clipped, and one embolized. Also, one partially resolved patient underwent a second embolization. No severe complications occurred in either group. Conclusions Despite the small number of patients, our study suggests that both surgical clipping and embolization are safe and effective methods in regards to functional recovery (complete ONP recovery in about 85 % of the cases). However, coiling may lead to delayed recurrence of third cranial nerve (CN) palsy at long-term follow-up, requiring additional treatment.

Abstract BACKGROUND Vascular encasement by skull base chordomas can increase surgical risk and hinder completeness of resection. However, the evidence behind this remains anecdotic. OBJECTIVE To give a better portrayal of chordomas encasing vertebrobasilar arteries mainly in regard of surgical vascular risk and its impact on extent of resection. METHODS A retrospective cohort study comparing skull base chordomas with encasement (≥180o encirclement) of the vertebrobasilar arteries to a control group of skull base chordomas with intradural extension. Data gathered involved pre- and postoperative volumetric analysis of the tumor, degree of encasement of involved vessel, occurrence of complication, and survival data including progression-free survival (PFS) and overall survival (OS). RESULTS A total of 24 patients with vertebrobasilar encasement were included in the study and an equal number of control cases were randomly selected from the same time period, totalizing 48 patients. Lower clival tumors with condyle involvement were more likely to have encasement. Gross total resection (GTR) rate was significantly lower in the encasement group (13% vs 42%, P = .023). Rates of postoperative new neurological deficit, CFS leak and 30 d postoperative mortality were not statistically different between groups. There was no statistically significant difference in mean PFS (P = .608) and OS (P = .958). CONCLUSION Skull base chordomas encasing vertebrobasilar arteries are highly challenging tumors. This study demonstrates that although safe resection is possible, GTR is hindered by the presence of encasement. We advocate letting the tumor's adherence to vessels lead the resection, leaving a small piece of tumor behind if adherent to the vessels.

Abstract BACKROUND Increasing indications for endoscopic endonasal approaches have led neurosurgeons to develop new reconstruction techniques for larger skull base defects. Vascularized grafts have been a great adjunction to reduce the rate of cerebrospinal fluid leak and can also be used to cover exposed critical structures such as the internal carotid artery. The nasoseptal flap and the inferior or middle turbinate flap are thus widely used in endoscopic skull base surgery, but may be insufficient for very large defects. OBJECTIVE To present a new mucosal flap used to cover large skull base defects in which the mucosa of the inferior turbinate, inferior meatus, nasal floor, and nasal septum is harvested in 1 piece keeping both vascular pedicles intact (inferior turbinate and septal arteries). METHODS We describe a surgical technique to harvest a combined inferior turbinate-nasoseptal flap. RESULTS Technical pearls and surgical pitfalls are described through 2 clinical cases in which the nasoseptal mucosa was partially damaged during a previous surgery, rendering the nasoseptal flap insufficient by itself. The flap is harvested thanks to 2 mucosal cuts: a first circular cut around the choanal arch and the junction between the hard and the soft palate, and a second one combining classical cuts of the nasoseptal flap and the inferior turbinate flap. CONCLUSION The inferior turbinate-nasoseptal flap can be a useful alternative in patients whose septal mucosa was partially damaged and/or with very large postoperative skull base defects.

BackgroundThe relationship between increased meningioma incidence and growth and long-term hormonal therapy with cyproterone acetate (CPA) in women has been recently established in literature. Following the raise in awareness from hormonal treatment, we describe a potential relationship between the progesterone agonist nomegestrol acetate (NOMAC) and meningioma growth.MethodsAfter implementation of a screening protocol to detect potential interactions between hormonal exposure and occurrence of meningioma, we identified patients taking NOMAC and newly diagnosed with a meningioma. NOMAC was stopped and those patients were followed tightly both clinically and radiologically. Retrospective volumetric analysis of the tumors was performed on the imaging.ResultsThree patients were identified for the study. After cessation of the NOMAC, tumor shrinkage was documented for all meningiomas within the first month. Up to 70% of tumor volume reduction was observed during the first year of follow-up in one of them. None of the patients developed new symptoms.ConclusionWe report the first cases of meningiomas responsiveness to discontinuation of hormonal therapy with NOMAC. Similarly to cases associated with long-term CPA intake, tumor reduction, and improvement of clinical symptoms can be observed after cessation of NOMAC.