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"Dumbbell" tumors are described as benign neoplasms presenting both intraspinal and extraspinal extensions, connected through the intervertebral foramen (McCormick, 1996) [1]. About 90 % of such tumors are histologically classified as schwannomas that most frequently arise in the thoracic region (Takamura and et al., 1997) [2]. Diagnosis is usually achieved as soon as the dimensional increase of the intracanal portion results in nerve or spinal cord compression (Ishikawa and et al., 2002) [3]. How to obtain a complete surgical resection of tumors with large or ventrally located extraforaminal components with a minimally invasive approach is still debated (Payer and et al., 2006) [4]. The single-stage posterior removal of the tumor is the most performed approach for lesions presenting with a small extra-foraminal component (Payer and et al., 2006) [4]. However, due to the reduced visual surgical field and poor control of the surrounding structures that could be obtained with an operative microscopic (OM) view, the application of this approach still appears to be limited to lesions with a large extraspinal component. An alternative surgical approach is the lateral transthoracic transpleural approach, which, however, carries greater risks of complications and often requires assistance from a thoracic surgeon. During the last decade, the exoscope was developed as a hybrid optical instrument, standing between the OM and the endoscope, merging the pros and cons of both visualization technologies, providing a wide viewing angle, high-resolution images, and non-monoaxial view. In this work we present a case of a 60-years old male patient with a 6-month history of dorsal pain and mild left limb paresthesia resistant to conservative treatment in which for the first time a single stage exoscopic-assisted (Olympus ORBEYE 4K-3D exoscope) posterior approach was used to remove entirely a thoracic dumbbell schwannoma with large extraspinal involvement.
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Background: Sporadic Spinal Psammomatous Malignant Melanotic Nerve Sheath Tumor (SSP-MMNST) is a rare subgroup of peripheral nerve sheath tumors arising along the spine. Only a few reports of SSP-MMNST have been described. In this paper, we review the literature on SSP-MMNST focusing on clinical, and diagnostic features, as well as investigating possible pathogenetic mechanisms to better implement therapeutic strategies. We also report an illustrative case of a young female presenting with cervicobrachial pain due to two SSP-MMNSTs arising from C5-6 right spinal roots. Case description: We report a case of a 28-year-old woman presenting with right arm weakness and dysesthesia. Clinical examination and neuroimaging were performed, and, following surgical removal of both lesions, a histological diagnosis of SSP-MMNST was obtained. Results: The literature review identified 21 eligible studies assessing 23 patients with SSP-MMNST, with a mean onset age of 41 years and a slight male gender preference. The lumbar district was the most involved spinal segment. Gross-total resection (GTR) was the treatment of choice in all amenable cases, followed in selected cases with residual tumor by adjuvant radiotherapy or chemotherapy. The metastatic and recurrence rates were 31.58% and 36.8%, respectively. Conclusion: Differently from common schwannomas, MMNST represents a rare disease with known recurrence and metastatization propensity. As reported in our review, SSP-MMNST has a greater recurrence rate when compared to other forms of spinal MMNST, raising questions about the greater aggressiveness of the former. We also found that residual disease is related to a higher risk of systemic disease spreading. This metastatic potential, usually associated with primary lumbar localization, is characterized by a slight male prevalence. Indeed, whenever GTR is unachievable, considering the higher recurrence rate, adjuvant radiation therapy should be taken into consideration.
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Meningiomas are uncommon in children and usually arise in the context of tumor-predisposing syndromes. Recently, YAP1-fusions have been identified for the first time as potential NF2-independent oncogenic drivers in the development of meningiomas in pediatric patients. We report a case of a YAP1-fusion-positive atypical meningioma in a young child and compare it with the previous ones reported. Extending the clinico-pathological features of YAP1-fused meningiomas, we suggest additional clues for diagnosis and emphasize the urgent need for an integrated multilayered diagnostic approach, combining data from histological and molecular analyses, neuroradiology, and clinical findings.
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Angioleiomyoma (ALM) is a benign smooth muscle neoplasm that mainly occurs in lower extremities subcutaneous tissue and generally affects middle-aged adults. This tumor histotype may rarely localize intracranially, although only a few cases have been described in the literature. We report a case of intracranial ALM, whose differential diagnosis has been particularly challenging, and firstly provide a comprehensive radiological and intra-operative evaluation of a such rare entity. This represents also the first report of the use of intraoperative confocal microscopy in ALM and the first documented short-term recurrence. At this regard, a scoping literature review has been conducted with the aim of presenting the major clinical and diagnostic features along with the proposed therapeutic strategies.
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The frontal aslant tract (FAT) is a recently identified white matter tract connecting the supplementary motor complex and lateral superior frontal gyrus to the inferior frontal gyrus. Advancements in neuroimaging and refinements to anatomical dissection techniques of the human brain white matter contributed to the recent description of the FAT anatomical and functional connectivity and its role in the pathogenesis of several neurological, psychiatric, and neurosurgical disorders. Through the application of diffusion tractography and intraoperative electrical brain stimulation, the FAT was shown to have a role in speech and language functions (verbal fluency, initiation and inhibition of speech, sentence production, and lexical decision), working memory, visual-motor activities, orofacial movements, social community tasks, attention, and music processing. Microstructural alterations of the FAT have also been associated with neurological disorders, such as primary progressive aphasia, post-stroke aphasia, stuttering, Foix-Chavany-Marie syndrome, social communication deficit in autism spectrum disorders, and attention-deficit hyperactivity disorder. We provide a systematic review of the current literature about the FAT anatomical connectivity and functional roles. Specifically, the aim of the present study relies on providing an overview for practical neurosurgical applications for the pre-operative, intra-operative, and post-operative assessment of patients with brain tumors located around and within the FAT. Moreover, some useful tests are suggested for the neurosurgical evaluation of FAT integrity to plan a safer surgery and to reduce post-operative deficits.
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Spinal Tuberculosis in children is uncommon, even more so in cases of involvement of posterior vertebral elements, and its diagnosis is often delayed. Here we report the case of a young female presenting neuroradiological features and clinical symptoms suspicious for malignant tumor. Histological examination of biopsy specimen evidenced a Pott's disease. We highlight the importance of suspecting this disorder in children with both aspecific systemic and neurological symptoms, in order to reach a timely diagnosis for appropriate and targeted intervention, avoiding the risk of overtreatment and malpractice claims.
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Neoplasias de la Columna Vertebral/diagnóstico por imagen , Neoplasias de la Columna Vertebral/patología , Tuberculosis de la Columna Vertebral/diagnóstico por imagen , Tuberculosis de la Columna Vertebral/patología , Niño , Errores Diagnósticos , Femenino , Humanos , Columna Vertebral/diagnóstico por imagen , Columna Vertebral/patologíaRESUMEN
INTRODUCTION: Atlanto-axial rotatory fixation (AARF) is a rare complication of ventriculo-peritoneal shunt (VPS) surgery. CASE PRESENTATION: The authors present a unique case of AARF developing early after VP shunting, with persistent torticollis, a "cock-robin" head position, and a thick fibrous band along the catheter path. Due to refractoriness to conservative treatments, AARF, which can be an early-onset complication of VPS surgery, was resolved by removing the distal catheter along with the fibrous band encasing it. CONCLUSION: Surgical removal of the fibrous band might be enough to solve such complication with no need of further surgical fusion procedures.
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Articulación Atlantoaxoidea/patología , Complicaciones Posoperatorias/etiología , Tortícolis/etiología , Derivación Ventriculoperitoneal/efectos adversos , Astrocitoma/complicaciones , Astrocitoma/cirugía , Neoplasias Encefálicas/complicaciones , Neoplasias Encefálicas/cirugía , Niño , Fibrosis/etiología , Humanos , Hidrocefalia/etiología , Hidrocefalia/cirugía , MasculinoRESUMEN
BACKGROUND: Glioblastoma (GBM) represents the most aggressive malignant brain tumor in adults, with a risible median life expectancy despite gold standard treatment. Novel drug-delivery methods have been explored. Here we evaluated the possibility to use mononuclear cells (MCs) belonging to the monocytic-dendritic lineage as drug-carrier. METHODS: MCs were obtained from 10 patients harboring a GBM, and from healthy volunteers, considered as controls. GBM tissue was also obtained from patients. MCs were cultured and the adherent population on fibronectin (FN-MCs), after immunocytochemistry and flow cytometry characterization, was loaded with Paclitaxel (FN-MCs-PTX). Antiproliferative and migration activity of FN-MCs-PTX was evaluated in two-dimensional (2D) and three-dimensional (3D) co-culture assays with red fluorescent U87 Malignant Glioma cells and primary GBM cells. Antiangiogenic properties of FN-MCs-PTX were tested on cultures with endothelial cells. RESULTS: Phenotypical characterization showed a high expression of monocytic-dendritic markers in GBM cells and FN-MCs. FN-MCs demonstrated to effectively uptake PTX and to strongly inhibit GBM growth in vitro (P <0.01). Moreover, tumor-induced migration of MCs, although partially affected by the PTX cargo, remained statistically significant when compared with unprimed cells and this was confirmed in a 3D Matrigel model (P <0.01) and in a Trans-well assay (P <0.01). FN-MCs-PTX also disclosed considerable antiangiogenic properties. DISCUSSION: Our results suggest that the fibronectin-adherent population of MCs isolated from peripheral blood can be an effective tool to inhibit GBM growth. Given the relative facility to obtain such cells and the short time needed for their culture and drug loading this approach may have potential as an adjuvant therapy for GBM.
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Antineoplásicos Fitogénicos/administración & dosificación , Neoplasias Encefálicas/tratamiento farmacológico , Sistemas de Liberación de Medicamentos/métodos , Fibronectinas/metabolismo , Glioblastoma/tratamiento farmacológico , Paclitaxel/administración & dosificación , Adulto , Anciano , Adhesión Celular , Línea Celular Tumoral , Técnicas de Cocultivo , Portadores de Fármacos , Humanos , Leucocitos Mononucleares/citología , Persona de Mediana EdadRESUMEN
BACKGROUND: Stereoelectroencephalography (SEEG) methodology, originally developed by Talairach and Bancaud, is progressively gaining popularity for the presurgical invasive evaluation of drug-resistant epilepsies. OBJECTIVE: To describe recent SEEG methodological implementations carried out in our center, to evaluate safety, and to analyze in vivo application accuracy in a consecutive series of 500 procedures with a total of 6496 implanted electrodes. METHODS: Four hundred nineteen procedures were performed with the traditional 2-step surgical workflow, which was modified for the subsequent 81 procedures. The new workflow entailed acquisition of brain 3-dimensional angiography and magnetic resonance imaging in frameless and markerless conditions, advanced multimodal planning, and robot-assisted implantation. Quantitative analysis for in vivo entry point and target point localization error was performed on a sub--data set of 118 procedures (1567 electrodes). RESULTS: The methodology allowed successful implantation in all cases. Major complication rate was 12 of 500 (2.4%), including 1 death for indirect morbidity. Median entry point localization error was 1.43 mm (interquartile range, 0.91-2.21 mm) with the traditional workflow and 0.78 mm (interquartile range, 0.49-1.08 mm) with the new one (P < 2.2 × 10). Median target point localization errors were 2.69 mm (interquartile range, 1.89-3.67 mm) and 1.77 mm (interquartile range, 1.25-2.51 mm; P < 2.2 × 10), respectively. CONCLUSION: SEEG is a safe and accurate procedure for the invasive assessment of the epileptogenic zone. Traditional Talairach methodology, implemented by multimodal planning and robot-assisted surgery, allows direct electrical recording from superficial and deep-seated brain structures, providing essential information in the most complex cases of drug-resistant epilepsy.
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Electroencefalografía , Epilepsia/cirugía , Procedimientos Neuroquirúrgicos/métodos , Técnicas Estereotáxicas , Cirugía Asistida por Computador/métodos , Adolescente , Adulto , Algoritmos , Encéfalo/cirugía , Angiografía Cerebral , Niño , Preescolar , Resistencia a Medicamentos , Electrodos Implantados/efectos adversos , Electroencefalografía/efectos adversos , Epilepsia/diagnóstico , Femenino , Humanos , Imagenología Tridimensional , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Análisis Multivariante , Robótica , Seguridad , Adulto JovenRESUMEN
We describe the 1-year follow-up after epilepsy surgery of 12 patients with chronic interictal psychosis and medically intractable epilepsy. The 12 cases described were part of a sample of 350 subjects who concluded a 1-year psychiatric follow-up from a total of 504 subjects operated on for medically intractable epilepsy between 2002 and 2009. Outcome was Engel class IA for five of these patients, IB for one, ID for one, IIB for one, IIIA for three, and IVA for one. Four patients had a worsening of psychotic symptoms at 6 months, but showed improvement after 1 year; two subjects had a worsening of psychotic symptoms at both 6 months and 1 year. Despite the severity of symptoms, compliance with preoperative assessment, surgical programs, and follow-up was satisfactory.