Afin de faciliter la dissection du nerf facial lors de l'exerese d'un neurinome de l'... more Afin de faciliter la dissection du nerf facial lors de l'exerese d'un neurinome de l'acoustique, les auteurs rapportent un procede faisant appel a une irrigation de serum pulsee qui facilite le clivage entre les deux elements. Ce procede semble minimiser la manipulation du tronc nerveux et, donc, le traumatisme chirurgical.
Purpose Management of optic nerve sheath meningiomas (ONSM) remains challenging. Photon radiation... more Purpose Management of optic nerve sheath meningiomas (ONSM) remains challenging. Photon radiation therapy (PhRT) is the most common treatment for sight-threatening ONSM. Proton beam therapy (PBT) is less commonly used because it is more expensive and because there are questions about its efficacy specifically in relation to ONSM. PBT has the theoretical advantage of reducing radiation exposure to adjacent structures. We report the visual outcome of patients with primary ONSM managed at the Fondation Ophtalmologique Adolphe de Rothschild, Paris, France, and treated with PBT at the Centre de Protonthérapie, Institut Curie, Orsay, France. Methods We conducted a retrospective review of all patients with primary ONSM who received PBT (either by itself or following surgery) between January 2006 and January 2019. Neuro-ophthalmic examinations were performed at presentation and after radiotherapy, and, when applicable, after surgery. Meningiomas were measured at the time of diagnosis and at...
BACKGROUND Recent discussions on the relationship between intracranial aneurysm diameter and rupt... more BACKGROUND Recent discussions on the relationship between intracranial aneurysm diameter and rupture probability have focused on the presence of an apparent critical diameter of 10 mm for aneurysm rupture. Despite the fact that many ...
A 41-year-old female presented in April 1996 with a tumor of the hard palate revealed by increasi... more A 41-year-old female presented in April 1996 with a tumor of the hard palate revealed by increasing left palate pain. Adenoid cystic carcinoma was suspected on clinical and imaging data. Two limited surgical procedures showed a tumor histologically made of small lobules of granular cells, PAS positive and expressing S100 protein, infiltrating some medullary spaces of the palatine bone, consistent with a granular-cell tumour. Pain recurred in the territory of the maxillary branch of the left trigeminal nerve (V2). Imaging showed a tumor of the origin of V2-extending through the foramen rotondum. Two radical interventions in September and in October 2000 showed an infiltrating tumor of the V2 and palatine mucosa, with the same histology. There was no immuno-staining for p53, and less than 5% of nuclei expressed Ki67. Malignant Abrikossof tumors are exceptional, morphologically difficult to differentiate from benign ones, only metastasis proving malignancy. Tumor size above 5 cm, recur...
Numerous molecular alterations have been described in supratentorial high-grade gliomas (1p19q co... more Numerous molecular alterations have been described in supratentorial high-grade gliomas (1p19q co-deletion, IDH1/2, histone H3, hTERT promotor mutations, loss of ATRX) which have led to a new histomolecular classification of diffuse gliomas. We aimed at describing these alterations in a series of 19 adults with pure cerebellar high-grade gliomas. Systematic immunohistochemical analyses, including that of IDH1R132H, ATRX, p53, PTEN, EGFR, p16, FGFR3, BRAFV600E, mismatch repair proteins, H3K27me3, H3K36me3, and H3K27M; molecular analyses of IDH1/2, hTERT, BRAF, H3F3A, and HIST1H3B mutation hotspots; and EGFR, PTEN FISH were retrospectively performed in a multicentric study. We histopathologically identified 14 glioblastomas, 4 grade III astrocytomas and 1 gliosarcoma. Two cases showed a H3F3A K27M mutation. Only one case harbored a classical profile of glioblastoma with hTERT mutation, EGFR gain and 10q loss. The most frequent alteration was the absence of p16 immunoexpression. We report a histomolecular analysis of pure cerebellar high grade gliomas. The histomolecular profile appears to be different from that of supratentorial gliomas, with no IDH1/2 gene mutations and only 1 case with a classic profile of de novo glioblastoma. In 2 cases, we identified H3F3A K27M mutation, classically described in pediatric midline gliomas. .
BACKGROUND Predictors of visual outcomes after optic nerve decompression are controversial. OBJEC... more BACKGROUND Predictors of visual outcomes after optic nerve decompression are controversial. OBJECTIVE To identify the predictors of poor visual outcomes after surgery of meningiomas responsible of a compressive optic neuropathy. METHODS We focused on paraoptic meningiomas (POMs), which gathered tuberculum sellae meningiomas (TSMs) and anterior clinoid meningiomas (ACMs) responsible for visual impairment or threatening visual function, that underwent surgery at our institution between January 2009 and December 2015 and analyzed the clinical and radiological findings of our patients. RESULTS Among 112 patients who underwent surgery for a POM, a preoperative visual deficit was present in 108 patients (96.4%). Six months after surgery, 79 patients (70.5%) had a visual improvement, 15 patients (13.4%) had an unchanged vision, and 18 patients (16.1%) had deteriorated vision. A preoperative visual deficit of 6 mo or more was a strong predictor of poor visual outcome after surgery (P = .034...
Since the outbreak of coronavirus disease 2019 (COVID-19) in December 2019 in China, various meas... more Since the outbreak of coronavirus disease 2019 (COVID-19) in December 2019 in China, various measures have been adopted in order to attenuate the impact of the virus on the population. With regard to spine surgery, French physicians are devoted to take place in the national plan against COVID-19, the French Spine Surgery Society therefore decided to elaborate specific guidelines for management of spinal disorders during COVID-19 pandemic in order to prioritize management of patients. A three levels stratification was elaborated with Level I: Urgent surgical indications, Level II: Surgical indications associated to a potential loss of chance for the patient and Level III: Non-urgent surgical indications. We also report French experience in a COVID-19 cluster region illustrated by two clinical cases. We hope that the guidelines formulated by the French Spine Surgery Society and the experience of spine surgeons from a cluster region will be helpful in order optimizing the management of patients with urgent spinal conditions during the pandemic.
We report the case of a 30-year-old woman with histologically proven monostotic fibrous dysplasia... more We report the case of a 30-year-old woman with histologically proven monostotic fibrous dysplasia of C2 revealed by a pathological fracture of the odontoid process. Radiological investigations showed a ground-glass mineralization of the vertebral body, a centimetric lytic area with poorly defined margins involving the inferior part of the vertebral body and inferior endplate and a fracture through an osteolytic area in the base of the odontoid process. Owing to the vertebral instability, a surgical procedure combining C0–C5 fixation and posterior bone grafting was performed. The surgical biopsy was inconclusive and pathological confirmation was finally obtained through a percutaneous needle biopsy under fluoroscopic guidance. At 26-month follow-up, the patient still experienced mild persistent cervical posterior neck pain and stiffness possibly related to a C5–6 laxity below the intervertebral fixation. This case combines three radiological findings, which are unusual in fibrous dys...
BACKGROUND The transoral approach provides the most direct exposure to extradural lesions of the ... more BACKGROUND The transoral approach provides the most direct exposure to extradural lesions of the ventral craniovertebral junction. The morbidity and mortality from this approach greatly limits its use because they are still feared and debated. Using univariable and multivariable logistic regression analyses, this study aims to identify the factors associated with short-term complications in patients undergoing the transoral approach. METHODS A consecutive cohort analysis of prospectively collected data in several neurosurgery spine departments evaluated 143 consecutive patients who had undergone craniovertebral junction transoral approach surgery. The mean age at the time of surgery was 45.1 ± 19.1 years. The study analyzed the comorbidities, the operative procedure, and postoperative morbidity and mortality. RESULTS Seventeen patients (11.9%) had a postoperative complication in the first month. In our univariable analysis: age, smoking, tumor etiology, preoperative posterior fixation, posterior bone graft, preoperative external lumbar shunt, and the transoral approach pathway were significantly associated with postoperative mortality and morbidity. In our multivariable analyses, preoperative external lumbar shunt was significantly associated with complication risks (odds ratio [OR] 6.7; 95% confidence interval [CI] 2.1-21.7, P < 0.001), whereas preoperative posterior fixation (OR 0.28; 95% CI 0.08-1.1, P < 0.04) and posterior bone graft (OR 0.14; 95% CI 0.03-0.6, P < 0.008) were significantly associated with lower complication risks. CONCLUSIONS To reduce complications, it is essential to be aware of and to manage these preoperative risk factors. In such manner, we insinuate that postoperative complications depend on the surgeon's familiarity with the transoral approach.
Malignant optic glioma of adulthood is a rare, invasive neoplasm of the anterior visual pathway. ... more Malignant optic glioma of adulthood is a rare, invasive neoplasm of the anterior visual pathway. In this article, the clinical features of a case series of 3 malignant optic nerve glioblastomas (World Health Organization grade IV) are presented, and the modalities of treatment and their associated survivals are discussed through a review of the existing literature to date. A retrospective case series study was led for 3 patients diagnosed with primary optic nerve and chiasm glioblastoma, coming from 2 referral neurosurgical centers. An electronic search was conducted on MEDLINE via PUBMED, COCHRANE, from October 1973 to April 2016. Cohort, case reports, and case series were screened for investigating treatment and overall survival (OS) of malignant optic nerve gliomas. Pooled means and 95% confidence intervals of OS for each treatment were generated. From our retrospective case series, all patients had initial visual impairment (2 women and 1 man). The histologic diagnosis was done by biopsy. The patients&#39; mean age was 67.3 years (standard deviation [SD] 18.5). The disease was rapidly lethal for all patients: median OS was 5 months (SD: 15.1). Two patients underwent chemotherapy by single cure of temozolomide, while the third one was treated with a radiochemotherapy protocol. Due to the fact that there is no gold standard treatment as first-choice treatment, a large heterogeneity in first-choice oncologic treatment is observed. However, we did not find any significant differences for OS between World Health Organization grade III and grade IV optic gliomas. Malignant optic glioma is a rare and fatal disease in adults. Despite the modalities of treatment, the treatment outcomes remain unsatisfactory. There is no significant difference in the median OS of patients with malignant optic nerve, as compared with those diagnosed with other supratentorial glioblastoma. Chemoradiotherapy with temozolomide currently remains the best treatment in terms of OS. Advances in the understanding of tumor biology have yet failed to translate into effective treatment regimens.
Brain pathology (Zurich, Switzerland), Jan 5, 2017
Clear cell meningioma (CCM) is a rare grade II histopathological subtype that usually occurs in y... more Clear cell meningioma (CCM) is a rare grade II histopathological subtype that usually occurs in young patients and displays high recurrence rate. Germline SMARCE1 mutations have been described in hereditary forms of this disease and more recently in small syndromic and sporadic CCM series. The diagnostic value of SMARCE1 in distinguishing between CCM and other meningioma variants has not been yet established. The aim of our study was to investigate the status of SMARCE1 in a series of CCMs and its morphological mimickers. We compared the performance of an anti-SMARCE1 antibody and the molecular analysis of the SMARCE1 gene in a retrospective multicenter series of CCMs. All CCMs lossed SMARCE1 immunoexpression. Bi-allelic inactivating events were found by NGS-based sequencing in all of these cases, except for one, which was incompletely explored, but had a wild-type sequence. We then validated the anti-SMARCE1 antibody specificity by analyzing additional 305 pediatric and adult menin...
OBJECTIVEThe aim of this study was to confirm the feasibility of an innovative transoral robotic ... more OBJECTIVEThe aim of this study was to confirm the feasibility of an innovative transoral robotic surgery (TORS), using the da Vinci Surgical System, for patients with sellar tumors. This technique was designed to offer a new minimally invasive approach, without soft-palate splitting, that avoids the rhinological side effects of classic endonasal approaches.METHODSThe authors performed a prospective study of TORS in patients with symptomatic sellar tumors. Specific anatomical features were required for inclusion in the study and were determined on the basis of preoperative open-mouth CT scans of the brain. The main outcome measure was sellar accessibility using the robot. Resection quality, mean operative time, postoperative changes in patients' vision, side effects, and complications were additionally reported.RESULTSBetween February and May 2016, 4 patients (all female, mean age 49.5 years) underwent TORS for resection of sellar tumors as participants in this study. All patient...
The prognosis of unruptured giant basilar artery (BA) aneurysms is very poor. No treatment has sh... more The prognosis of unruptured giant basilar artery (BA) aneurysms is very poor. No treatment has shown efficacy in survival. This pilot case-control study examines the overall survival (OS) benefit of combined surgical and endovascular management of giant BA aneurysms. Combined treatment including posterior fossa craniectomy followed by endovascular treatment was performed in 3 patients with giant BA aneurysms. OS of the 3 patients was compared with a control group of 6 patients (ratio 1:2) treated with the endovascular procedure only. The mean survival time was 32.6 months in the craniectomy group (SD 9.01, 95% confidence interval [14.9, 50.3]) and 3.5 months in the control group (SD = 2.08, 95% confidence interval [0.001, 7.6]; Mantel-Cox test P &lt; 0.04). At mean follow-up of 36.5 months (SD 10.2), 2 of 3 patients had a favorable outcome with a Glasgow Outcome Scale score of 5. Univariate analysis determined that women had a statistically higher OS than men (33.7 months vs. 3.058 months for men; log-rank test P = 0.011). A similar outcome was obtained in the presence of a circulating posterior communicating artery (P = 0.03) and in the presence of an endovascular right vertebral artery occlusion (P = 0.022). Our study suggests that preventive posterior fossa craniectomy increases significantly OS of patients with giant BA aneurysms.
Anaplastic gangliogliomas (GGGs) are rare tumors whose natural history is poorly documented. We a... more Anaplastic gangliogliomas (GGGs) are rare tumors whose natural history is poorly documented. We aimed to define their clinical and imaging features and to identify prognostic factors. Consecutive cases of anaplastic GGGs in adults prospectively entered into the French Brain Tumor Database between March 2004 and April 2014 were screened. After diagnosis was confirmed by pathological review, clinical, imaging, therapeutic, and outcome data were collected retrospectively. Forty-three patients with anaplastic GGG (median age, 49.4 y) from 18 centers were included. Presenting symptoms were neurological deficit (37.2%), epileptic seizure (37.2%), or increased intracranial pressure (25.6%). Typical imaging findings were unifocal location (94.7%), contrast enhancement (88.1%), central necrosis (43.2%), and mass effect (47.6%). Therapeutic strategy included surgical resection (95.3%), adjuvant radiochemotherapy (48.8%), or radiotherapy alone (27.9%). Median progression-free survival (PFS) and overall survival (OS) were 8.0 and 24.7 months, respectively. Three- and 5-year tumor recurrence rates were 69% and 100%, respectively. The 5-year survival rate was 24.9%. Considering unadjusted significant prognostic factors, tumor midline crossing and frontal location were associated with shorter OS. Temporal and parietal locations were associated with longer and shorter PFS, respectively. None of these factors remained statistically significant in multivariate analysis. We report a large series providing clinical, imaging, therapeutic, and prognostic features of adult patients treated for an intracerebral anaplastic GGG. Our results show that pathological diagnosis is difficult, that survivals are only slightly better than for glioblastomas, and that complete surgical resection followed with adjuvant chemoradiotherapy offers longer survival.
Afin de faciliter la dissection du nerf facial lors de l'exerese d'un neurinome de l'... more Afin de faciliter la dissection du nerf facial lors de l'exerese d'un neurinome de l'acoustique, les auteurs rapportent un procede faisant appel a une irrigation de serum pulsee qui facilite le clivage entre les deux elements. Ce procede semble minimiser la manipulation du tronc nerveux et, donc, le traumatisme chirurgical.
Purpose Management of optic nerve sheath meningiomas (ONSM) remains challenging. Photon radiation... more Purpose Management of optic nerve sheath meningiomas (ONSM) remains challenging. Photon radiation therapy (PhRT) is the most common treatment for sight-threatening ONSM. Proton beam therapy (PBT) is less commonly used because it is more expensive and because there are questions about its efficacy specifically in relation to ONSM. PBT has the theoretical advantage of reducing radiation exposure to adjacent structures. We report the visual outcome of patients with primary ONSM managed at the Fondation Ophtalmologique Adolphe de Rothschild, Paris, France, and treated with PBT at the Centre de Protonthérapie, Institut Curie, Orsay, France. Methods We conducted a retrospective review of all patients with primary ONSM who received PBT (either by itself or following surgery) between January 2006 and January 2019. Neuro-ophthalmic examinations were performed at presentation and after radiotherapy, and, when applicable, after surgery. Meningiomas were measured at the time of diagnosis and at...
BACKGROUND Recent discussions on the relationship between intracranial aneurysm diameter and rupt... more BACKGROUND Recent discussions on the relationship between intracranial aneurysm diameter and rupture probability have focused on the presence of an apparent critical diameter of 10 mm for aneurysm rupture. Despite the fact that many ...
A 41-year-old female presented in April 1996 with a tumor of the hard palate revealed by increasi... more A 41-year-old female presented in April 1996 with a tumor of the hard palate revealed by increasing left palate pain. Adenoid cystic carcinoma was suspected on clinical and imaging data. Two limited surgical procedures showed a tumor histologically made of small lobules of granular cells, PAS positive and expressing S100 protein, infiltrating some medullary spaces of the palatine bone, consistent with a granular-cell tumour. Pain recurred in the territory of the maxillary branch of the left trigeminal nerve (V2). Imaging showed a tumor of the origin of V2-extending through the foramen rotondum. Two radical interventions in September and in October 2000 showed an infiltrating tumor of the V2 and palatine mucosa, with the same histology. There was no immuno-staining for p53, and less than 5% of nuclei expressed Ki67. Malignant Abrikossof tumors are exceptional, morphologically difficult to differentiate from benign ones, only metastasis proving malignancy. Tumor size above 5 cm, recur...
Numerous molecular alterations have been described in supratentorial high-grade gliomas (1p19q co... more Numerous molecular alterations have been described in supratentorial high-grade gliomas (1p19q co-deletion, IDH1/2, histone H3, hTERT promotor mutations, loss of ATRX) which have led to a new histomolecular classification of diffuse gliomas. We aimed at describing these alterations in a series of 19 adults with pure cerebellar high-grade gliomas. Systematic immunohistochemical analyses, including that of IDH1R132H, ATRX, p53, PTEN, EGFR, p16, FGFR3, BRAFV600E, mismatch repair proteins, H3K27me3, H3K36me3, and H3K27M; molecular analyses of IDH1/2, hTERT, BRAF, H3F3A, and HIST1H3B mutation hotspots; and EGFR, PTEN FISH were retrospectively performed in a multicentric study. We histopathologically identified 14 glioblastomas, 4 grade III astrocytomas and 1 gliosarcoma. Two cases showed a H3F3A K27M mutation. Only one case harbored a classical profile of glioblastoma with hTERT mutation, EGFR gain and 10q loss. The most frequent alteration was the absence of p16 immunoexpression. We report a histomolecular analysis of pure cerebellar high grade gliomas. The histomolecular profile appears to be different from that of supratentorial gliomas, with no IDH1/2 gene mutations and only 1 case with a classic profile of de novo glioblastoma. In 2 cases, we identified H3F3A K27M mutation, classically described in pediatric midline gliomas. .
BACKGROUND Predictors of visual outcomes after optic nerve decompression are controversial. OBJEC... more BACKGROUND Predictors of visual outcomes after optic nerve decompression are controversial. OBJECTIVE To identify the predictors of poor visual outcomes after surgery of meningiomas responsible of a compressive optic neuropathy. METHODS We focused on paraoptic meningiomas (POMs), which gathered tuberculum sellae meningiomas (TSMs) and anterior clinoid meningiomas (ACMs) responsible for visual impairment or threatening visual function, that underwent surgery at our institution between January 2009 and December 2015 and analyzed the clinical and radiological findings of our patients. RESULTS Among 112 patients who underwent surgery for a POM, a preoperative visual deficit was present in 108 patients (96.4%). Six months after surgery, 79 patients (70.5%) had a visual improvement, 15 patients (13.4%) had an unchanged vision, and 18 patients (16.1%) had deteriorated vision. A preoperative visual deficit of 6 mo or more was a strong predictor of poor visual outcome after surgery (P = .034...
Since the outbreak of coronavirus disease 2019 (COVID-19) in December 2019 in China, various meas... more Since the outbreak of coronavirus disease 2019 (COVID-19) in December 2019 in China, various measures have been adopted in order to attenuate the impact of the virus on the population. With regard to spine surgery, French physicians are devoted to take place in the national plan against COVID-19, the French Spine Surgery Society therefore decided to elaborate specific guidelines for management of spinal disorders during COVID-19 pandemic in order to prioritize management of patients. A three levels stratification was elaborated with Level I: Urgent surgical indications, Level II: Surgical indications associated to a potential loss of chance for the patient and Level III: Non-urgent surgical indications. We also report French experience in a COVID-19 cluster region illustrated by two clinical cases. We hope that the guidelines formulated by the French Spine Surgery Society and the experience of spine surgeons from a cluster region will be helpful in order optimizing the management of patients with urgent spinal conditions during the pandemic.
We report the case of a 30-year-old woman with histologically proven monostotic fibrous dysplasia... more We report the case of a 30-year-old woman with histologically proven monostotic fibrous dysplasia of C2 revealed by a pathological fracture of the odontoid process. Radiological investigations showed a ground-glass mineralization of the vertebral body, a centimetric lytic area with poorly defined margins involving the inferior part of the vertebral body and inferior endplate and a fracture through an osteolytic area in the base of the odontoid process. Owing to the vertebral instability, a surgical procedure combining C0–C5 fixation and posterior bone grafting was performed. The surgical biopsy was inconclusive and pathological confirmation was finally obtained through a percutaneous needle biopsy under fluoroscopic guidance. At 26-month follow-up, the patient still experienced mild persistent cervical posterior neck pain and stiffness possibly related to a C5–6 laxity below the intervertebral fixation. This case combines three radiological findings, which are unusual in fibrous dys...
BACKGROUND The transoral approach provides the most direct exposure to extradural lesions of the ... more BACKGROUND The transoral approach provides the most direct exposure to extradural lesions of the ventral craniovertebral junction. The morbidity and mortality from this approach greatly limits its use because they are still feared and debated. Using univariable and multivariable logistic regression analyses, this study aims to identify the factors associated with short-term complications in patients undergoing the transoral approach. METHODS A consecutive cohort analysis of prospectively collected data in several neurosurgery spine departments evaluated 143 consecutive patients who had undergone craniovertebral junction transoral approach surgery. The mean age at the time of surgery was 45.1 ± 19.1 years. The study analyzed the comorbidities, the operative procedure, and postoperative morbidity and mortality. RESULTS Seventeen patients (11.9%) had a postoperative complication in the first month. In our univariable analysis: age, smoking, tumor etiology, preoperative posterior fixation, posterior bone graft, preoperative external lumbar shunt, and the transoral approach pathway were significantly associated with postoperative mortality and morbidity. In our multivariable analyses, preoperative external lumbar shunt was significantly associated with complication risks (odds ratio [OR] 6.7; 95% confidence interval [CI] 2.1-21.7, P < 0.001), whereas preoperative posterior fixation (OR 0.28; 95% CI 0.08-1.1, P < 0.04) and posterior bone graft (OR 0.14; 95% CI 0.03-0.6, P < 0.008) were significantly associated with lower complication risks. CONCLUSIONS To reduce complications, it is essential to be aware of and to manage these preoperative risk factors. In such manner, we insinuate that postoperative complications depend on the surgeon's familiarity with the transoral approach.
Malignant optic glioma of adulthood is a rare, invasive neoplasm of the anterior visual pathway. ... more Malignant optic glioma of adulthood is a rare, invasive neoplasm of the anterior visual pathway. In this article, the clinical features of a case series of 3 malignant optic nerve glioblastomas (World Health Organization grade IV) are presented, and the modalities of treatment and their associated survivals are discussed through a review of the existing literature to date. A retrospective case series study was led for 3 patients diagnosed with primary optic nerve and chiasm glioblastoma, coming from 2 referral neurosurgical centers. An electronic search was conducted on MEDLINE via PUBMED, COCHRANE, from October 1973 to April 2016. Cohort, case reports, and case series were screened for investigating treatment and overall survival (OS) of malignant optic nerve gliomas. Pooled means and 95% confidence intervals of OS for each treatment were generated. From our retrospective case series, all patients had initial visual impairment (2 women and 1 man). The histologic diagnosis was done by biopsy. The patients&#39; mean age was 67.3 years (standard deviation [SD] 18.5). The disease was rapidly lethal for all patients: median OS was 5 months (SD: 15.1). Two patients underwent chemotherapy by single cure of temozolomide, while the third one was treated with a radiochemotherapy protocol. Due to the fact that there is no gold standard treatment as first-choice treatment, a large heterogeneity in first-choice oncologic treatment is observed. However, we did not find any significant differences for OS between World Health Organization grade III and grade IV optic gliomas. Malignant optic glioma is a rare and fatal disease in adults. Despite the modalities of treatment, the treatment outcomes remain unsatisfactory. There is no significant difference in the median OS of patients with malignant optic nerve, as compared with those diagnosed with other supratentorial glioblastoma. Chemoradiotherapy with temozolomide currently remains the best treatment in terms of OS. Advances in the understanding of tumor biology have yet failed to translate into effective treatment regimens.
Brain pathology (Zurich, Switzerland), Jan 5, 2017
Clear cell meningioma (CCM) is a rare grade II histopathological subtype that usually occurs in y... more Clear cell meningioma (CCM) is a rare grade II histopathological subtype that usually occurs in young patients and displays high recurrence rate. Germline SMARCE1 mutations have been described in hereditary forms of this disease and more recently in small syndromic and sporadic CCM series. The diagnostic value of SMARCE1 in distinguishing between CCM and other meningioma variants has not been yet established. The aim of our study was to investigate the status of SMARCE1 in a series of CCMs and its morphological mimickers. We compared the performance of an anti-SMARCE1 antibody and the molecular analysis of the SMARCE1 gene in a retrospective multicenter series of CCMs. All CCMs lossed SMARCE1 immunoexpression. Bi-allelic inactivating events were found by NGS-based sequencing in all of these cases, except for one, which was incompletely explored, but had a wild-type sequence. We then validated the anti-SMARCE1 antibody specificity by analyzing additional 305 pediatric and adult menin...
OBJECTIVEThe aim of this study was to confirm the feasibility of an innovative transoral robotic ... more OBJECTIVEThe aim of this study was to confirm the feasibility of an innovative transoral robotic surgery (TORS), using the da Vinci Surgical System, for patients with sellar tumors. This technique was designed to offer a new minimally invasive approach, without soft-palate splitting, that avoids the rhinological side effects of classic endonasal approaches.METHODSThe authors performed a prospective study of TORS in patients with symptomatic sellar tumors. Specific anatomical features were required for inclusion in the study and were determined on the basis of preoperative open-mouth CT scans of the brain. The main outcome measure was sellar accessibility using the robot. Resection quality, mean operative time, postoperative changes in patients' vision, side effects, and complications were additionally reported.RESULTSBetween February and May 2016, 4 patients (all female, mean age 49.5 years) underwent TORS for resection of sellar tumors as participants in this study. All patient...
The prognosis of unruptured giant basilar artery (BA) aneurysms is very poor. No treatment has sh... more The prognosis of unruptured giant basilar artery (BA) aneurysms is very poor. No treatment has shown efficacy in survival. This pilot case-control study examines the overall survival (OS) benefit of combined surgical and endovascular management of giant BA aneurysms. Combined treatment including posterior fossa craniectomy followed by endovascular treatment was performed in 3 patients with giant BA aneurysms. OS of the 3 patients was compared with a control group of 6 patients (ratio 1:2) treated with the endovascular procedure only. The mean survival time was 32.6 months in the craniectomy group (SD 9.01, 95% confidence interval [14.9, 50.3]) and 3.5 months in the control group (SD = 2.08, 95% confidence interval [0.001, 7.6]; Mantel-Cox test P &lt; 0.04). At mean follow-up of 36.5 months (SD 10.2), 2 of 3 patients had a favorable outcome with a Glasgow Outcome Scale score of 5. Univariate analysis determined that women had a statistically higher OS than men (33.7 months vs. 3.058 months for men; log-rank test P = 0.011). A similar outcome was obtained in the presence of a circulating posterior communicating artery (P = 0.03) and in the presence of an endovascular right vertebral artery occlusion (P = 0.022). Our study suggests that preventive posterior fossa craniectomy increases significantly OS of patients with giant BA aneurysms.
Anaplastic gangliogliomas (GGGs) are rare tumors whose natural history is poorly documented. We a... more Anaplastic gangliogliomas (GGGs) are rare tumors whose natural history is poorly documented. We aimed to define their clinical and imaging features and to identify prognostic factors. Consecutive cases of anaplastic GGGs in adults prospectively entered into the French Brain Tumor Database between March 2004 and April 2014 were screened. After diagnosis was confirmed by pathological review, clinical, imaging, therapeutic, and outcome data were collected retrospectively. Forty-three patients with anaplastic GGG (median age, 49.4 y) from 18 centers were included. Presenting symptoms were neurological deficit (37.2%), epileptic seizure (37.2%), or increased intracranial pressure (25.6%). Typical imaging findings were unifocal location (94.7%), contrast enhancement (88.1%), central necrosis (43.2%), and mass effect (47.6%). Therapeutic strategy included surgical resection (95.3%), adjuvant radiochemotherapy (48.8%), or radiotherapy alone (27.9%). Median progression-free survival (PFS) and overall survival (OS) were 8.0 and 24.7 months, respectively. Three- and 5-year tumor recurrence rates were 69% and 100%, respectively. The 5-year survival rate was 24.9%. Considering unadjusted significant prognostic factors, tumor midline crossing and frontal location were associated with shorter OS. Temporal and parietal locations were associated with longer and shorter PFS, respectively. None of these factors remained statistically significant in multivariate analysis. We report a large series providing clinical, imaging, therapeutic, and prognostic features of adult patients treated for an intracerebral anaplastic GGG. Our results show that pathological diagnosis is difficult, that survivals are only slightly better than for glioblastomas, and that complete surgical resection followed with adjuvant chemoradiotherapy offers longer survival.
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