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BACKGROUND AND OBJECTIVES: Patients with basilar invagination (BI) can be treated with several surgical options, ranging from simple posterior decompression to circumferential decompression and fusion. Here, we aimed at examining the indications and outcomes associated with these surgical strategies to devise a staged algorithm for managing BI. METHODS: We conducted a retrospective cohort study in 2 neurosurgical centers and included patients with a BI, as defined by a position of the dens tip at least 5 mm above the Chamberlain line. Other craniovertebral junction anomalies, such as atlas assimilation, platybasia, and Chiari malformations, were documented. C1-C2 stability was assessed with a dynamic computed tomography scan. RESULTS: We included 30 patients with BI with a mean follow-up of 56 months (min = 12, max = 166). Posterior decompression and fusion (n = 8) was only performed in cases of obvious atlanto-axial instability (eg, increased atlanto-dental interval or hypermobility on flexion/extension), while anterior decompression (transoral or transnasal) was reserved to patients with lower cranial nerves deficits (eg, swallowing dysfunction) and irreducible anterior compression (n = 9). Patients with posterior signs (eg, Valsalva headaches) or myelopathy but without C1-C2 instability nor anterior signs were managed with an isolated foramen magnum decompression, with or without duraplasty (n = 13). Complications were more frequent for combined procedures, including neurological deterioriation (n = 4) and tracheostomy (n = 2), but reinterventions were more likely in patients undergoing posterior decompression alone (n = 3). CONCLUSION: Patient selection is key to determine the appropriate surgical strategy for BI: In our experience, combined approaches are only needed for patients with irreducible and symptomatic anterior compression, while fusion should be restricted to patient with obvious signs of atlanto-axial instability. Other BI patients can be managed by foramen magnum decompression alone to minimize surgical morbidity.
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Neoplasias Meníngeas , Meningioma , Neoplasias de la Base del Cráneo , Humanos , Meningioma/diagnóstico por imagen , Meningioma/cirugía , Foramen Magno/diagnóstico por imagen , Foramen Magno/cirugía , Neoplasias Meníngeas/diagnóstico por imagen , Neoplasias Meníngeas/cirugía , Nariz , Neoplasias de la Base del Cráneo/diagnóstico por imagen , Neoplasias de la Base del Cráneo/cirugíaRESUMEN
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 each follow-up MRI examination. RESULTS: Sixty patients (50 women, 10 men; mean age, 45.2±11.1y) were included, of whom 29 underwent surgery. At presentation, 52 (87%) of them had decreased vision (average visual acuity: 0.6 logMAR). Fundus examination showed optic disc swelling (n=27; 46.5%), optic disc pallor (n=22; 37.9%), optic disc cupping (n=2; 3.4%), opto-ciliary shunt (n=8; 13.8%), or choroidal folds (n=5; 8.6%). Otherwise, it was unremarkable (n=7; 12.1%). After treatment, visual function was stable overall. Fundus examination showed pallor (n=47; 83.9%), swelling (n=3; 5.4%), or cupping (n=2; 3.4%) of the optic disc, or was unremarkable (n=5; 8.9%). The visual field of 8 patients worsened, while 3 developed asymptomatic retinal hemorrhages. Tumor shrunk significantly in 8 patients at 1 year after PBT and remained stable in size in all others. Patients with opto-ciliary shunts had significantly worse visual outcome than other patients. Retinal abnormalities were observed in 11 patients during follow-up. CONCLUSION: PBT alone or in association with surgery appears to be a safe and efficient treatment for ONSM, reducing the tumor size and stabilizing visual function. The risk of developing radiation retinopathy seems to be higher when patients had upfront surgery.
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PURPOSE: Myxopapillary ependymoma (MPE) is the most frequent tumor affecting the medullary conus. The surgical therapeutic management is still debated and only few studies have focused on the postoperative clinical outcome of patients. This study aimed to demonstrate long-term postoperative outcome and to assess the predictive factors of recurrence as well as the clinical evolution of these patients. METHODS: From 1984 to 2019, in four French centers, 101 adult patients diagnosed with MPE were retrospectively included. RESULTS: Median age at surgery was 39 years. Median tumor size was 50 mm and lesions were multifocal in 13% of patients. All patients benefited from surgery and one patient received postoperative radiotherapy. Gross total resection was obtained in 75% of cases. Sixteen percent of patients presented recurrence after a median follow-up of 70 months. Progression free survival at 5 and 10 years were respectively estimated at 83% and 79%. After multivariable analysis, sacral localization, and subtotal resection were shown to be independently associated with tumor recurrence. 85% of the patients had a favorable evolution concerning pain. 12% of the patients presented a postoperative deterioration of sphincter function and 4% of motor function. CONCLUSION: Surgery alone is an acceptable option for MPE patients. Patients with sacral location or incomplete resection are at high risk of recurrence and should be carefully monitored.
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Ependimoma , Neoplasias de la Médula Espinal , Ependimoma/cirugía , Humanos , Estudios Retrospectivos , Neoplasias de la Médula Espinal/cirugía , Resultado del TratamientoRESUMEN
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). Poor visual outcome after surgery was not significantly related to the size of the tumor (P = .057), the age of the patient (P = .94), or the tumor extension into the optic canal (P = .47). CONCLUSION: The duration of preoperative visual deficit was found to be a strong predictor of poor visual outcomes after surgery in POMs Other predictors of poor visual outcomes are still needed and are currently under evaluation in a prospective study at our institution.
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Neoplasias Meníngeas , Meningioma , Humanos , Neoplasias Meníngeas/complicaciones , Neoplasias Meníngeas/diagnóstico por imagen , Neoplasias Meníngeas/cirugía , Meningioma/complicaciones , Meningioma/diagnóstico por imagen , Meningioma/cirugía , Estudios Prospectivos , Estudios Retrospectivos , Silla Turca , Resultado del TratamientoRESUMEN
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.
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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 dysplasia: monostotic presentation involving the spine, some aggressive radiographic features, and a pathological fracture.
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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.â©.
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Neoplasias Cerebelosas/genética , Neoplasias Cerebelosas/patología , Glioma/genética , Glioma/patología , Histonas/genética , Neoplasias Supratentoriales/genética , Neoplasias Supratentoriales/patología , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Femenino , Perfilación de la Expresión Génica , Humanos , Inmunohistoquímica , Masculino , Persona de Mediana Edad , Mutación/genética , Proteínas de Neoplasias/genética , Estudios Retrospectivos , Adulto JovenRESUMEN
BACKGROUND: Neurologic manifestations of craniovertebral junction (CVJ) disease may generate dramatic brainstem damage, which could evolve to paralysis. In most cases, patients are referred with advanced neurological symptoms such as tetraplegia/paresis. The aim of this study was to identify predictive factors of favorable neurological evolution after non-traumatic brainstem compression. METHODS: A prospective study evaluated 143 consecutive patients who had undergone CVJ anterior brainstem decompression. The mean age was 45.1 ± 19.1 years. The study analyzed clinical, surgical, and imagery characters to determine predictive factors of neurological improvement. RESULTS: The mean follow-up of our series was 10.2 years (range 0.5-23.9). Seventy-one (49.6%) presented initial tetrapalsies resulting from spinal cord compression. Multivariable analysis revealed that Frankel score [odds ratio (OR) 5.7, CI 95% 1.01-31.8; p < 0.04] and preoperative symptoms < 6 months [OR 0.33, CI 95% 0.125-0.9; p < 0.025] were independently associated with partial neurological improvement, while the only independent factor associated with total neurologic recovery was the preoperative symptom evolution <6 months [odd ratio (OR) 4.3, CI 95% 1.6-11.4; p < 0.003]. None of the following were identified as predictive factors: demographic characteristics, medical history, the etiology of compression, or initial spinal cord MRI. CONCLUSION: The earlier the decompression is performed, the better the neurological improvement. Whatever the initial Frankel score, if neurological palsy or disorders evolved for less than 6 months, complete recovery is possible.
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Tronco Encefálico/cirugía , Descompresión Quirúrgica/efectos adversos , Complicaciones Posoperatorias/epidemiología , Compresión de la Médula Espinal/cirugía , Adulto , Anciano , Tronco Encefálico/diagnóstico por imagen , Tronco Encefálico/patología , Descompresión Quirúrgica/métodos , Femenino , Humanos , Masculino , Persona de Mediana Edad , Complicaciones Posoperatorias/prevención & control , Compresión de la Médula Espinal/diagnóstico por imagen , Compresión de la Médula Espinal/patologíaRESUMEN
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.
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Vértebras Cervicales/cirugía , Procedimientos Ortopédicos/métodos , Complicaciones Posoperatorias/diagnóstico , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Vértebras Cervicales/diagnóstico por imagen , Niño , Preescolar , Comorbilidad , Femenino , Estudios de Seguimiento , Humanos , Masculino , Persona de Mediana Edad , Complicaciones Posoperatorias/etiología , Complicaciones Posoperatorias/mortalidad , Pronóstico , Estudios Prospectivos , Estudios Retrospectivos , Factores de Riesgo , Adulto JovenRESUMEN
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 meningiomas of various subtypes and 15 non-meningioma clear cell tumors by SMARCE1 immunohistochemistry. A nuclear immunostaining was preserved in all other meningioma variants, as well as non-meningioma clear cell tumors. In conclusion, our series showed, for the first time, that SMARCE1 immunostaining is a highly sensitive biomarker for CCM, useful as a routine diagnostic biomarker.
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Proteínas Cromosómicas no Histona/genética , Proteínas Cromosómicas no Histona/metabolismo , Proteínas de Unión al ADN/genética , Proteínas de Unión al ADN/metabolismo , Neoplasias Meníngeas , Meningioma , Adolescente , Adulto , Anciano , Anticuerpos/administración & dosificación , Biomarcadores de Tumor/metabolismo , Niño , Proteínas Cromosómicas no Histona/inmunología , Proteínas de Unión al ADN/inmunología , Femenino , Genotipo , Humanos , Inmunohistoquímica , Masculino , Neoplasias Meníngeas/diagnóstico , Neoplasias Meníngeas/genética , Neoplasias Meníngeas/metabolismo , Meningioma/diagnóstico , Meningioma/genética , Meningioma/metabolismo , Persona de Mediana Edad , Estudios Retrospectivos , Adulto JovenRESUMEN
Background: 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. Methods: 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. Results: 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. Conclusions: 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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Neoplasias Encefálicas/patología , Terapia Combinada/mortalidad , Ganglioglioma/patología , Adolescente , Adulto , Anciano , Neoplasias Encefálicas/terapia , Bases de Datos Factuales , Progresión de la Enfermedad , Femenino , Estudios de Seguimiento , Ganglioglioma/terapia , Humanos , Masculino , Persona de Mediana Edad , Pronóstico , Estudios Retrospectivos , Tasa de Supervivencia , Adulto JovenRESUMEN
BACKGROUND: 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. METHODS: 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. RESULTS: 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 < 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). CONCLUSIONS: Our study suggests that preventive posterior fossa craniectomy increases significantly OS of patients with giant BA aneurysms.
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Fosa Craneal Posterior/cirugía , Craniectomía Descompresiva/métodos , Procedimientos Endovasculares/métodos , Aneurisma Intracraneal/cirugía , Anciano , Anciano de 80 o más Años , Estudios de Casos y Controles , Fosa Craneal Posterior/diagnóstico por imagen , Femenino , Humanos , Aneurisma Intracraneal/diagnóstico por imagen , Aneurisma Intracraneal/mortalidad , Angiografía por Resonancia Magnética , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Proyectos Piloto , Estudios Retrospectivos , Estadísticas no Paramétricas , Tomógrafos Computarizados por Rayos XRESUMEN
OBJECTIVE The 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. METHODS The 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. RESULTS Between 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 patients presented with symptomatic visual deficits confirmed as bitemporal hemianopsia. All tumors had a suprasellar portion and a cystic part. In all 4 cases, the operation was performed via TORS, without the need for a second surgery. Sella turcica accessibility was satisfactory in all cases. In 3 cases, tumor resection was complete. The mean operative time was 2 hours 43 minutes. Three patients had a significant visual improvement at Day 1. No rhinological side effects or complications in patients occurred. No pathological examination was performed regarding the fluid component of the tumors. There was 1 postoperative delayed CSF leak and 1 case of transient diabetes insipidus. Side effects specific to TORS included minor sore throat, transient hypernasal speech, and 1 case of delayed otitis media. The mean length of hospital stay and mean follow up were 8.25 days and 82 days, respectively. CONCLUSIONS To our knowledge, this is the first report of the surgical treatment of sellar tumors by means of a minimally invasive TORS. This approach using the da Vinci Surgical System seems feasible and constitutes an innovative neurosurgical technique that may avoid the adverse side effects and technical disadvantages of the classic transsphenoidal route. Moreover, TORS allows an inferosuperior approach to the sella turcica, which is a key point, as the tumor is approached in the direction of its growth.
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Procedimientos Quirúrgicos Robotizados/métodos , Silla Turca , Neoplasias Craneales/cirugía , Adulto , Estudios de Factibilidad , Femenino , Humanos , Persona de Mediana Edad , Boca , Estudios Prospectivos , Tomografía Computarizada por Rayos XRESUMEN
BACKGROUND: 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. METHODS: 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. RESULTS: 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' 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. CONCLUSION: 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.
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Glioma/diagnóstico , Glioma/terapia , Neoplasias del Nervio Óptico/diagnóstico , Neoplasias del Nervio Óptico/terapia , Trastornos de la Visión/prevención & control , Anciano , Anciano de 80 o más Años , Resultado Fatal , Femenino , Glioma/complicaciones , Humanos , Masculino , Persona de Mediana Edad , Neoplasias del Nervio Óptico/complicaciones , Pronóstico , Trastornos de la Visión/etiologíaRESUMEN
BACKGROUND: Multiple spinal hemangioblastomas constitute a rare pathology; most patients have von Hippel-Lindau disease. In the literature, spinal surgery has been complicated with remote intracranial hemorrhage in a very few cases. METHODS: A 51-year-old woman, with no pertinent familial past medical history, presented with low back pain. Medullary magnetic resonance imaging revealed 3 spinal extramedullary tumors. The tumor at the L4 level appeared as a dumbbell-shaped neurinoma-like mass with enlarged vessels nearby. Medullary angiography excluded a dural fistula. Surgery was performed in 2 stages for all lesions. The day after lumbar surgery, the patient became comatose because of a remote cerebellar hemorrhage associated with hydrocephalus. She underwent external ventricular drainage and posterior fossa craniectomy. RESULTS: The patient recovered fully at 35 days except for a slight disorientation and diplopia. Histology of all lesions revealed hemangioblastomas. Discussion of this case concerns radiologic features of spinal hemangioblastomas, diagnosis of von Hippel-Lindau disease, and pathophysiology of hemorrhagic complications following spinal surgery. CONCLUSIONS: This case report includes 2 rare entities, of which every spinal surgeon should be aware.
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Hemorragia Cerebral/etiología , Hemangioblastoma/cirugía , Procedimientos Neuroquirúrgicos/efectos adversos , Neoplasias de la Columna Vertebral/cirugía , Enfermedad de von Hippel-Lindau/cirugía , Hemorragia Cerebral/patología , Femenino , Hemangioblastoma/patología , Humanos , Persona de Mediana Edad , Neoplasias de la Columna Vertebral/patología , Enfermedad de von Hippel-Lindau/patologíaRESUMEN
PURPOSE: To detect the presence of hypoxic tissue, which is known to increase the radioresistant phenotype, by its uptake of fluoromisonidazole (18F) (FMISO) using hybrid positron emission tomography/computed tomography (PET/CT) imaging, and to compare it with the glucose-avid tumor tissue imaged with fluorodeoxyglucose (18F) (FDG), in residual postsurgical skull base chordoma scheduled for radiotherapy. PATIENTS AND METHODS: Seven patients with incompletely resected skull base chordomas were planned for high-dose radiotherapy (dose ≥70 Gy). All 7 patients underwent FDG and FMISO PET/CT. Images were analyzed qualitatively by visual examination and semiquantitatively by computing the ratio of the maximal standardized uptake value (SUVmax) of the tumor and cerebellum (T/C R), with delineation of lesions on conventional imaging. RESULTS: Of the eight lesion sites imaged with FDG PET/CT, only one was visible, whereas seven of nine lesions were visible on FMISO PET/CT. The median SUVmax in the tumor area was 2.8 g/mL (minimum 2.1; maximum 3.5) for FDG and 0.83 g/mL (minimum 0.3; maximum 1.2) for FMISO. The T/C R values ranged between 0.30 and 0.63 for FDG (median, 0.41) and between 0.75 and 2.20 for FMISO (median,1.59). FMISO T/C R >1 in six lesions suggested the presence of hypoxic tissue. There was no correlation between FMISO and FDG uptake in individual chordomas (r = 0.18, p = 0.7). CONCLUSION: FMISO PET/CT enables imaging of the hypoxic component in residual chordomas. In the future, it could help to better define boosted volumes for irradiation and to overcome the radioresistance of these lesions. No relationship was founded between hypoxia and glucose metabolism in these tumors after initial surgery.
Asunto(s)
Cordoma/diagnóstico por imagen , Fluorodesoxiglucosa F18 , Misonidazol/análogos & derivados , Imagen Multimodal/métodos , Tomografía de Emisión de Positrones , Radiofármacos , Neoplasias de la Base del Cráneo/diagnóstico por imagen , Tomografía Computarizada por Rayos X , Adulto , Hipoxia de la Célula , Cordoma/metabolismo , Cordoma/radioterapia , Femenino , Fluorodesoxiglucosa F18/farmacocinética , Humanos , Masculino , Persona de Mediana Edad , Misonidazol/farmacocinética , Recurrencia Local de Neoplasia , Estudios Prospectivos , Radiofármacos/farmacocinética , Neoplasias de la Base del Cráneo/metabolismo , Neoplasias de la Base del Cráneo/radioterapia , Adulto JovenRESUMEN
STUDY DESIGN: Retrospective study. OBJECTIVE: To assess outcomes after lumboperitoneal shunting for CES with dural sac dilation and multiple arachnoid diverticula in patients with long-standing AS. SUMMARY OF BACKGROUND DATA: Cauda equina syndrome (CES) is a rare complication of long-standing ankylosing spondylitis (AS). Neurologic symptoms occur insidiously and have a poor prognosis without effective treatment. METHODS: We retrospectively studied cases seen between 1997 and 2009 at our university-hospital rheumatology department. RESULTS: We identified 9 patients with AS and dural sac dilation (mean age: 64, range: 38-75), of whom 8 experienced CES 29.1 years on average (range: 10-51) after AS onset. Presenting symptoms were urinary abnormalities (n = 4), sensory abnormalities (n = 6), or radicular or low back pain (n = 4). The symptoms worsened progressively, with mild (n = 3) to severe (n = 1) motor deficiency, sphincter dysfunction (urinary [n = 6] and/or anal [n = 4] sphincter), and impotence (n = 3). Magnetic resonance imaging showed dural sac dilation (n = 9), multiple lumbar arachnoid diverticula (n = 6), erosions of the laminae and spinous processes (n = 6), and nerve-root tethering (n = 6) with adhesion to the dura mater and vertebrae (n = 7). Cerebrospinal fluid (CSF) flow study by magnetic resonance imaging was performed in 2 patients and showed communication of the diverticula with the CSF. Lumboperitoneal shunting, performed in 5 patients, was followed by improvements in sensation (n = 4), urinary symptoms (n = 2), anal continence (n = 3), and pain. Full recovery of muscle strength was noted in 3 patients. Improvements persisted after a mean of 49 months (range: 18-96). CONCLUSION: Lumboperitoneal shunting induced substantial improvements in all 5 patients treated with this procedure. This result suggests that AS-related CES may be due to chronic arachnoiditis and dural fibrosis leading to diminished CSF resorption with dural sac dilation and diverticula formation.