Prognosis of atypical teratoid rhabdoid tumors (AT/RT) treated with multimodal therapy protocols. Report of our series.

OBJECTIVE: Atypical teratoid rhabdoid tumors (AT/RT) of the central nervous system are rare, very aggressive embryological tumors, typically diagnosed in young patients and having a low survival rate after diagnosis. The aim of this study was to emphasize, based on the latest results in the literature, the need for protocols for multidisciplinary treatment in these patients. MATERIAL AND METHODS: We report our series of 3 cases treated, diagnosed and followed up between 2009 and 2014. They were treated with multimodal therapy protocols (Rhabdoid SIOP-2007 and European Rhabdoid Registry EU-RHAB-2010). In addition, we carried out a literature review. RESULTS: Two of our 3 cases (supratentorial and spinal tumors) did not show any progression of the disease after long follow-up, in contrast with most of the cases available in the literature. The second patient had a shorter survival. CONCLUSIONS: Patient age at the time of diagnosis, supratentorial location of the mass and fewer complications with adjuvant treatments seem to be factors yielding good prognosis for AT/RT tumors. In agreement with the latest international protocols, multidisciplinary treatment is the ideal treatment, consisting of radiotherapy and chemotherapy after complete tumor resection.

[Choroid plexus tumour treatment at Hospital Infantil Niño Jesús in Madrid: Our experience over the last three decades]. / Tratamiento de los tumores de plexos coroideos en el Hospital Infantil Niño Jesús de Madrid: nuestra experiencia en las últimas tres décadas.

OBJECTIVE: To review childhood patients with choroid plexus tumors (CPT) who underwent surgery at Hospital Infantil Niño Jesús of Madrid since January 1981 to September 2014. MATERIAL AND METHODS: Registered charts were analyzed based on the epidemiology, tumor grade, clinical profile, location, dissemination characteristics, therapy, prognosis and complications. RESULTS: Seventeen childhood patients were recorded with CPT. Cases were distributed so that 9 cases were choroid plexus-papilloma (CPP) (52.9%), 2 cases atypical CPP (11.7%) and 6 cases choroid plexus-carcinoma (CPC) (35.2%). Age at diagnosis was less than 2 years in 14 of the 17 patients (82.3%) and the incidence was higher in males (82.3% of the cases). Gross total resection was performed in 16 patients (94.1%). Adjuvant treatment was used in 6 patients (all this cases with CPC) (35.2%). Two of the 17 patients died (11.7%), showing an incidence density of 0.01 deaths/year. CONCLUSIONS: Our case series is consistent with previous published in scientific literature regarding epidemiology, tumor grade, clinical presentation, radiological features and therapeutic approach. Gross total resection is considered the therapeutic gold standard for choroid plexus tumors. Chemotherapy and radiotherapy should be used as adjuvant treatment in CPC and recurrent or remaining atypical CPP.

[Congenital medulloblastoma associated with intracranial arachnoid cyst]. / Meduloblastoma congénito asociado a quiste aracnoideo intracraneal.

Arachnoid cysts are very common lesions in paediatric patients, with treatment depending on their location and symptomatology. They are usually solitary cysts but may be associated with other central nervous system diseases such as tumours and congenital deformities. We describe the case of a neonate diagnosed with an arachnoid cyst of the quadrigeminal cistern treated by endoscopy. After the operation, the child's condition worsened; a CT scan revealed a midline posterior fossa tumour not visible in the preoperative neuroradiological tests. The tumour, a medulloblastoma, was partially removed. Given the child's age and the poor prognosis, oncological treatment was not undertaken. The association between medulloblastoma and arachnoid cyst is very rare, and we could find only one such case in the literature.

Fatigue in patients with acquired brain damage.

Fatigue is a complex, multidimensional syndrome that is prevalent in patients with acquired brain damage and has a negative impact on the neurorehabilitation process. It presents from early stages after the injury, and may persist over time, regardless of whether sequelae have resolved. Fatigue is conditioned by upper neuronal circuits, and is defined as an abnormal perception of overexertion. Its prevalence ranges from 29% to 77% after stroke, from 18% to 75% after traumatic brain injury, and from 47% to 97% after brain tumours. Fatigue is associated with factors including female sex, advanced age, dysfunctional families, history of specific health conditions, functional status (eg, fatigue prior to injury), comorbidities, mood, secondary disability, and the use of certain drugs. Assessment of fatigue is fundamentally based on such scales as the Fatigue Severity Scale (FSS). Advances have recently been made in imaging techniques for its diagnosis, such as in functional MRI. Regarding treatment, no specific pharmacological treatment currently exists; however, positive results have been reported for some conventional neurorehabilitation therapies, such as bright light therapy, neurofeedback, electrical stimulation, and transcranial magnetic stimulation. This review aims to assist neurorehabilitation professionals to recognise modifiable factors associated with fatigue and to describe the treatments available to reduce its negative effect on patients.

[Early magnetic resonance imaging detection of a cavernous angioma after cranial radiotherapy for an anaplastic ependymoma in a boy]. / Detección precoz mediante resonancia magnética de un angioma cavernoso tras radioterapia craneal por ependimoma anaplásico en un niño.

Radiotherapy forms part of most therapeutic, preventive, and conditioning regimens in pediatric oncology. Numerous late secondary effects of cranial radiation are well known. However, radiation-induced cavernous angiomas (RICA) have been reported only sporadically and even fewer cases of earlier presentation of RICA have been reported. In this brief report, we describe a RICA that appeared in a boy treated for a CNS tumor (an infratentorial anaplastic ependymoma) after a short latency period between the end of radiotherapy and the development of the RICA. We comment on the different variables proposed to explain the formation of these lesions, as well as on their imaging features, treatment, prognosis, and follow-up.

Hemorrhagic presentation of previously silent brain tumors.

INTRODUCTION AND OBJECTIVES: Acute presentation with intracranial hemorrhage owing to a previously silent brain tumor (BT) is rare. Although any BT can bleed, the frequency and type of bleeding varies across tumor types. MATERIALS AND METHODS: We aimed to retrospectively review our experience with 55 patients with BTs presenting with ICH. RESULTS: Signs of increased intracranial pressure were the most common symptoms. The temporal lobe was the most common lesion site (n=22). Hemorrhages were mainly confined to the tumor margins (HCTs) (n=34). Extensive intraparenchymal hemorrhages (EIHs) were mainly associated with moderately/severely decreased levels of consciousness (LOCs) (n=15/16). High-grade glioma (HGGT) (n=25) was the leading pathological diagnosis followed by metastasis (MBT) (n=16/55). The hemorrhage type was associated with the pathological diagnosis of the tumor. Patients with HGGT (n=19/25) and MBT (n=9/16) mainly presented with HCTs, whereas low-grade gliomas (LGGT) primarily caused EIHs (n=6/7). CONCLUSIONS: Hemorrhagic presentation is a rare occurrence in BTs. Among all, MBT and HGGT are responsible for majority of the cases. Importantly, despite their relatively benign characteristics, LGGTs mainly result in extensive parenchymal destruction once they bleed. Maximum surgical resection of hemorrhagic BTs and decompression of the affected brain regions followed by histological confirmation of the diagnosis should be the main goals of treatment in cases with hemorrhagic BTs.

Low grade gliomas guide-lines elaborated by the tumor section of Spanish Society of Neurosurgery.

Adult low-grade gliomas (Low Grade Gliomas, LGG) are tumors that originate from the glial cells of the brain and whose management involves great controversy, starting from the diagnosis, to the treatment and subsequent follow-up. For this reason, the Tumor Group of the Spanish Society of Neurosurgery (GT-SENEC) has held a consensus meeting, in which the most relevant neurosurgical issues have been discussed, reaching recommendations based on the best scientific evidence. In order to obtain the maximum benefit from these treatments, an individualised assessment of each patient should be made by a multidisciplinary team. Experts in each LGG treatment field have briefly described it based in their experience and the reviewed of the literature. Each area has been summarized and focused on the best published evidence. LGG have been surrounded by treatment controversy, although during the last years more accurate data has been published in order to reach treatment consensus. Neurosurgeons must know treatment options, indications and risks to participate actively in the decision making and to offer the best surgical treatment in every case.

Brain metastasis treatment guidelines: consensus by the Spanish Society of Neurosurgery Tumor Section.

Brain metastases are tumors that arise from a tumor cell originated in another organ reaching the brain through the blood. In the brain this tumor cell is capable of growing and invading neighboring tissues, such as the meninges and bone. In most patients a known tumor is present when the brain lesion is diagnosed, although it is possible that the first diagnose is the brain tumor before there is evidence of cancer elsewhere in the body. For this reason, the neurosurgeon must know the management that has shown the greatest benefit for brain metastasis patients, so treatments can be streamlined and optimized. Specifically, in this document, the following topics will be developed: selection of the cancer patient candidate for surgical resection and the role of the neurosurgeon in the multidisciplinary team, the importance of immunohistological and molecular diagnosis, surgical techniques, radiotherapy techniques, treatment updates of chemotherapy and immunotherapy and management algorithms in brain metastases. With this consensus manuscript, the tumor group of the Spanish Society of Neurosurgery (GT-SENEC) exposes the most relevant neurosurgical issues and the fundamental aspects to harmonize multidisciplinary treatment, especially with the medical specialties that are treating or will treat these patients.

Myxofibrosarcoma: Another mimicker of meningioma.

Primary intracranial malignant fibrous histiocytoma (MFH), or myxofibrosarcoma, is an extremely rare condition, with only a few cases reported in the literature. We report a case of a dural-based myxofibrosarcoma in a previously healthy 42-year-old man that was initially presumed to be an atypical meningioma. The findings based on conventional and advanced magnetic resonance sequences, including diffusion-weighted imaging, perfusion weighted imaging and proton magnetic resonance spectroscopy, as well as histopathological aspects, are discussed.

The effects of altered neurogenic microRNA levels and their involvement in the aggressiveness of periventricular glioblastoma.

INTRODUCTION: Glioblastoma multiforme is the most common primary brain tumour, with the least favourable prognosis. Despite numerous studies and medical advances, it continues to be lethal, with an average life expectancy of 15 months after chemo-radiotherapy. DEVELOPMENT: Recent research has addressed several factors associated with the diagnosis and prognosis of glioblastoma; one significant factor is tumour localisation, particularly the subventricular zone, which represents one of the most active neurogenic niches of the adult human brain. Glioblastomas in this area are generally more aggressive, resulting in unfavourable prognosis and a shorter life expectancy. Currently, the research into microRNAs (miRNA) has intensified, revealing different expression patterns under physiological and pathophysiological conditions. It has been reported that the expression levels of certain miRNAs, mainly those related to neurogenic processes, are dysregulated in oncogenic events, thus favouring gliomagenesis and greater tumour aggressiveness. This review discusses some of the most important miRNAs involved in subventricular neurogenic processes and their association with glioblastoma aggressiveness. CONCLUSIONS: MiRNA regulation and function play an important role in the development and progression of glioblastoma; understanding the alterations of certain miRNAs involved in both differentiation and neural and glial maturation could help us to better understand the malignant characteristics of glioblastoma.

Primary intracranial melanoma, amelanotic variant: Case report.

Primary brain melanoma is a very rare tumour (only 0.07% of primary CNS neoplasms) which usually shows with abundant melanin content; whereas hypo/melanotic variants have been scarcely described. We introduce the case of a female patient with headache, left brachial paresis and frontallobar syndrome. The MRI image showed a right frontal mass with homogeneous contrast uptake. As treatment, a complete surgical resection was performed. Pathology was diagnostic for melanoma, with very low melanin content and a high proliferative index. A thorough extension study was performed to rule out an extracranial primary origin. Due to several intercurrent complications, the patient evolved unfavorably, not being able to receive further treatment. The amelanotic variant of primary intracranial malignant melanomas has not been described in detail previously. We will review the literature, focusing on the particularities of management and diagnosis of this clinical entity.

Primary central nervous system Hodgkin lymphoma: A case report and review of the literature.

Central nervous system (CNS) involvement in the context of relapsed/refractory Hodgkin lymphoma (HL) is a quite rare, but well-known complication. Nevertheless, primary CNS-HL is an exceedingly rare condition, which diagnosis is based on well-defined morphological and immunohistochemical features, in addition to isolated involvement of the CNS. In spite of limited casuistry (just over twenty cases reported in the literature), available data agree that primary and isolated CNS-HL, when treated with a combination of surgery followed by some form of adjuvant therapy (radiotherapy±chemotherapy), carries a better prognosis than those cases with CNS involvement in the context of relapsed/refractory HL or those with CNS non-Hodgkin lymphoma. We herein report a case of a 55-year-old female patient who was diagnosed with primary CNS-HL. The patient was treated with complete surgical resection followed by intrathecal chemotherapy and whole brain radiotherapy (WBRT), showing fourteen months of disease-free survival at the time of this case report. A review of the available literature is also presented.

Preoperative 3D volume reconstruction of the posterior wall of the sphenoid sinus with Horos: A free, simple and reliable tool in endoscopic endonasal trans-sphenoidal surgery.

BACKGROUND: The purpose of the study was to create computer-aided design models of the sphenoid sinus with a free-source software in order to perform a preoperative planning during trans-sphenoidal endoscopic surgery (TES) and clarify the three-dimensional anatomical features of the sphenoid sinus and its surrounding structures. METHODS: For each patient a 3D volume rendering of the sphenoid sinus was obtained from a thin slice head and maxilla-facial CT scan using a free-source DICOM viewer. The 3D models obtained preoperatively were examined preoperatively by six neurosurgeons in order to identify the boundaries of the sella. RESULTS: For the main anatomical landmark, all of the observers were able to recognize the anatomical structure at a rate ranging from 80 to 98%, 28 to 60% and 25 to 58% for expert (n=3), inexpert (n=3) and the entire group of observers (n=6), respectively. The analysis of the data shows that both observation groups presented a lower recognition rate of the following parameters: right and left medial and lateral optocarotid recesses and tumor prominence, however, the sellar prominence, clival recess, planum sphenoidalis, right and left ICA prominence, right and left optic prominences represent the main anatomical landmarks to be recognized during TES immediately before the opening of the sellar floor. CONCLUSIONS: The use of a preoperative 3D imaging is not in itself a novelty in the literature, however the fact that a simple tool obtained with a free-source software like Horos can represent a considerable help in surgical practice without resorting to the use of more complex software and expensive represents the real utility of this work.

[Lipomatous ependymomas of the posterior fossa. An infrequent and little-known subtype. A case report and review of the literature]. / Ependimomas lipomatosos de la fosa posterior. Un subtipo muy infrecuente y poco conocido. Presentación de caso y revisión de la literatura.

Ependymomas are well defined glial tumours composed of uniform small cells with round nuclei in a fibrillar matrix. They have characteristic perivascular acellular areas (pseudorosettes) and, in some cases, ependymal rosettes. The three most well-known histological phenotypes are papillary, clear-cell and tanycytic. The WHO classification includes rare cases of ependymoma with lipomatous metaplasia. Lipomatous ependymomas of the posterior fossa are extremely rare; we only found 7reports of cases in adults. They usually arise in the fourth ventricle and may extend into the cerebellum, when they often show extensive vacuolization, pushing the nucleus to the periphery and giving rise to a signet-ring cell appearance. Radiologically, there are few findings characteristic of these tumours. Immunohistochemistry is essential to differentiate this subtype from other more common lesions, such as metastatic adenocarcinoma, especially from breast, intestine and kidney.

Stereotactic biopsy for intracranial space occupying lesions: Experience in a referral hospital in Mexico.

BACKGROUND: Stereotactic brain biopsy (SBB) is used for establishing the histological diagnosis of intracranial lesions that are not amenable for a direct surgical approach. OBJECTIVE: The objective of the study was to describe our experience having an evaluation of the biopsy sample by a neuropathologist during SBB. MATERIALS AND METHODS: Retrospective analysis of 140 consecutive patients who underwent SBB between 2014 and 2018 in whom trans-operatory analysis of the sample was performed. RESULTS: There were 56% men. The mean age was 45 years. Histological diagnosis was performed in 131 of 140 patients (94% overall diagnostic yield). The presurgical radiological diagnosis was correct in 39%. Neoplastic lesions were reported in 108 cases, and 32 were non-neoplastic. We performed craniotomy and resection after biopsy in 14%. We found complications in 6% of patients. CONCLUSIONS: SBB continues to be a safe, useful, and inexpensive procedure. The diagnostic performance of SBB increases when intraoperative cytological evaluation by a neuropathologist is included in the study.

ANTECEDENTES: la biopsia cerebral por estereotaxia (SBB) se utiliza para establecer el diagnóstico histológico de lesiones intracraneales que no son susceptibles de un abordaje quirúrgico directo. OBJETIVO: describir nuestra experiencia de tener una evaluación de la muestra de biopsia por un neuropatólogo durante el procedimiento. MATERIAL Y MÉTODOS: análisis retrospectivo de 140 pacientes consecutivos sometidos a SBB entre 2014-2018 en los que se realizó análisis transoperatorio de la muestra. RESULTADOS: El 56% fueron hombres. La edad promedio fue de 45 años. El diagnóstico histológico se realizó en 131 de 140 pacientes (rendimiento diagnóstico global del 94%). El diagnóstico radiológico prequirúrgico fue correcto solo en el 39%. Se identificaron lesiones neoplásicas en 108 casos, y en 32 se documentaron lesiones no neoplásicas. En el 14% de los casos se realizó posterior a la biospia craneotomía y resección de la lesión. Encontramos complicaciones en el 6% de los pacientes. CONCLUSIONES: SBB sigue siendo un procedimiento seguro, útil y económico. El rendimiento diagnóstico de SBB aumenta cuando se incluye la evaluación citológica intraoperatoria por un neuropatólogo.

Results in the treatment of intracranial hemangiopericytomas. Case series. / Resultados en el tratamiento de los hemangiopericitomas intracraneanos. Serie de casos.

OBJECTIVE: Intracranial hemangiopericytoma (HPC) is a rare central nervous system tumor characterized by its low incidence, high rate of local recurrence and risk of metastasis. The main objectives of this paper are two: to show the results in the treatment of HPC in our institution in the last 20years and to make a review of the literature on this topic. METHODS: Retrospective review that includes patients diagnosed with intracranial solitary fibrous tumor/hemangiopericytoma (SFT/HPC) that have undergone surgery from 1997 to 2017. It includes patients that had nuclear expression of STAT-6 (detected by immunohistochemistry) and gradeII/III histopathological diagnosis (defined by the World Health Organization in 2016). We collected demographic data, tumor characteristics, treatment and survival of these patients. RESULTS: A total of 19 patients fulfilled inclusion criteria. The median follow up was 96 months (12-230). The mortality rate was 21% (n=4). 57.9% of patients presented at least one tumor recurrence (n=11) (recurrences of 6%, 67% y 90% at 1, 5 and 10years). Five patients presented extracranial metastasis. Patients with tumors <6cm had greater survival (P<.05). CONCLUSIONS: A series of patients undergoing SFT/HPC were presented according to the new WHO criteria. Size is a predictor of survival. Currently there are no validated criteria for surgical resection in this pathology. A classification with surgical guidance would be useful.

Fatigue in patients with acquired brain damage. / La fatiga en el daño cerebral sobrevenido.

Fatigue is a complex, multidimensional syndrome that is prevalent in patients with acquired brain damage and has a negative impact on the neurorehabilitation process. It presents from early stages after the injury, and may persist over time, regardless of whether sequelae have resolved. Fatigue is conditioned by upper neuronal circuits, and is defined as an abnormal perception of overexertion. Its prevalence ranges from 29% to 77% after stroke, from 18% to 75% after traumatic brain injury, and from 47% to 97% after brain tumours. Fatigue is associated with factors including female sex, advanced age, dysfunctional families, history of specific health conditions, functional status (eg, fatigue prior to injury), comorbidities, mood, secondary disability, and the use of certain drugs. Assessment of fatigue is fundamentally based on such scales as the Fatigue Severity Scale (FSS). Advances have recently been made in imaging techniques for its diagnosis, such as in functional MRI. Regarding treatment, no specific pharmacological treatment currently exists; however, positive results have been reported for some conventional neurorehabilitation therapies, such as bright light therapy, neurofeedback, electrical stimulation, and transcranial magnetic stimulation. This review aims to assist neurorehabilitation professionals to recognise modifiable factors associated with fatigue and to describe the treatments available to reduce its negative effect on patients.

Myxofibrosarcoma: Another mimicker of meningioma.

Primary intracranial malignant fibrous histiocytoma (MFH), or myxofibrosarcoma, is an extremely rare condition, with only a few cases reported in the literature. We report a case of a dural-based myxofibrosarcoma in a previously healthy 42-year-old man that was initially presumed to be an atypical meningioma. The findings based on conventional and advanced magnetic resonance sequences, including diffusion-weighted imaging, perfusion weighted imaging and proton magnetic resonance spectroscopy, as well as histopathological aspects, are discussed.

Related factors with diagnostic yield and intracranial hemorrhagic complications in frame-based stereotactic biopsy. Review.

The frame-based stereotactic biopsy is a minimally invasive technique that allows us to obtain a sample of brain tissue for subsequent diagnosis and treatment. The scope of this article is to review the published data related to the factors that could condition its diagnostic yield, and the appearance of post-biopsy hemorrhagic complications. PubMed search, last updated June 2020, was conducted using the terms "stereotactic biopsy", "diagnostic yield" and "intracranial post-biopsy hemorrhage". A total of 38 studies, that showed descriptive or analytical results, were included, and reviewed. Our literature review show that some characteristics of the lesion and surgical procedure peculiarities are significantly related with the effectiveness and safety of the technique. In this way, they must be taken into account in order to optimize its results.

Related factors with diagnostic yield and intracranial hemorrhagic complications in frame-based stereotactic biopsy. Review. / Factores condicionantes del rendimiento diagnóstico y las complicaciones hemorrágicas intracraneales en la biopsia estereotáctica con marco. Revisión.

The frame-based stereotactic biopsy is a minimally invasive technique that allows us to obtain a sample of brain tissue for subsequent diagnosis and treatment. The scope of this article is to review the published data related to the factors that could condition its diagnostic yield, and the appearance of post-biopsy hemorrhagic complications. PubMed search, last updated June 2020, was conducted using the terms "stereotactic biopsy", "diagnostic yield" and "intracranial post-biopsy hemorrhage". A total of 38 studies, that showed descriptive or analytical results, were included, and reviewed. Our literature review show that some characteristics of the lesion and surgical procedure peculiarities are significantly related with the effectiveness and safety of the technique. In this way, they must be taken into account in order to optimize its results.