Spinal metastases combined with leptomeningeal metastasis: A case report / 北京大学学报(医学版)

Spinal metastases (SM) is the commonest form of solid tumors osseous metastasis, for which surgical dissection is often performed when combined with spinal cord compression. Leptomeningeal metastasis (LM) results from dissemination of cancer cells to both the leptomeninges (pia and arachnoid) and cerebrospinal fluid (CSF) compartment. The spread of LM may occur via multiple routes, such as hematogenous, direct infiltration from metastatic brain lesions, or via iatrogenic seeding of CSF. Signs and symptoms associated with LM are generalized and various while early diagnosis of LM is challenging. Cytological evaluation of the CSF and gadolinium enhanced MRI brain and spine is the gold standard for diagnosing LM and CSF can help assess treatment response. While a number of other potential CSF biomarkers have been investigated both for the diagnosis as well as monitoring of LM, none have been established as a component of the standard evaluation of all LM or suspected LM patients. Management goals of LM include improving patient's neurologic function, quality of life, preventing further neurologic deterioration and prolonging survival. In many cases, it may be reasonable to pursue a palliative and comfort focused course, even from the initial LM diagnosis. Surgery is not recommended considering the risk of seeding with cerebrospinal fluid. A diagnosis of LM carries a poor prognosis with an estimated median survival of only 2-4 months despite therapy. Spinal metastases combined with leptomeningeal metastasis (SM+LM) is not uncommon and its treatment is similar to LM. LM can appear at the same time as SM or directly invaded by SM, which is thought regarding the pathophysiology of LM remains speculative and not systematically studied. The present article reports a 58-year-old woman who was first diagnosed with SM, but worsened after surgery repeated MRI examinations confirmed coexisting LM. Relevant literature was reviewed to summarize the epidemiology, clinical manifestations, imaging characteristics, diagnosis and treatment of SM+LM, so as to improve the understanding of the disease and promote early diagnosis. It should be vigilant to merge LM for the patient with SM when atypical clinical manifestations, rapid disease progression or inconsistent with imaging occurred. Repeated examinations of cerebrospinal fluid cytology and enhanced MRI should be considered when SM+LM is suspected to achieve timely adjustment of diagnosis and treatment strategy for better prognosis.

Advances in Diagnosis and Treatment of Leptomeningeal Metastasis of Lung Cancer / 中国肺癌杂志

Leptomeningeal metastases (LM), a special type of metastasis in advanced lung cancer, is known for its severe clinical symptoms, rapid progression and poor prognosis. LM used to be featured with low clinical diagnosis rate, limited treatment options, poor treatment efficacy, and very short survival if treatment not given. Though cerebrospinal fluid (CSF) cytology remains to be the gold standard for the diagnosis of LM, the positive rate of the first CSF cytology even in patients with suggestive clinical symptoms and positive imaging generally does not exceed 50%, leading to a delay in the diagnosis and treatment of patients with LM. With the progress of targeted therapy for driver gene-positive lung cancer and immunotherapy for driver gene-negative lung cancer, the overall survival of patients with lung cancer has been prolonged, meanwhile incidence of LM has been increasing year by year. Current clinical research in this field center around how to improve diagnosis rate and to find effective treatment approaches. This paper reviews advances in diagnosis and treatment of LM of lung cancer..
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Retrospective Analysis of Pathological Diagnosis of Central Nervous System Diseases in Tibet / 中国医学科学院学报

Objective To analyze the disease spectrum and clinicopathological characteristics of central nervous system(CNS)diseases diagnosed based on pathological findings in Tibet. Methods We collected the data of all the cases with CNS lesions in Tibet Autonomous Region People's Hospital from January 2013 to December 2020.The clinicopathological features were analyzed via light microscopy,immunohistochemical staining,and special staining. Results A total of 383 CNS cases confirmed by pathological diagnosis were enrolled in this study,with a male-to-female ratio of 188∶195 and an average age of(40.03±17.39)years(0-74 years).Among them,127(33.2%)cases had non-neoplastic diseases,with a male-to-female ratio of 82∶45 and an average age of(31.99±19.29)years;256(66.8%)cases had neoplastic diseases,with a male-to-female ratio of 106∶150 and an average age of(44.01±14.87)years.The main non-neoplastic diseases were nervous system infectious diseases,cerebral vascular diseases,meningocele,cerebral cyst,and brain trauma.Among the infectious diseases,brain abscess,granulomatous inflammation,cysticercosis,and hydatidosis were common.The main neoplastic diseases included meningioma,pituitary adenoma,neuroepithelial tumor,schwannoma,metastatic tumor,and hemangioblastoma.The meningioma cases consisted of 95.4%(103/108)cases of grade Ⅰ,3.7%(4/108)cases of grade Ⅱ,and only 1(1/108,0.9%)case of grade Ⅲ.Among the neuroepithelial tumor cases,the top three were glioblastoma,grade Ⅲ diffuse glioma,and ependymoma. Conclusions There are diverse CNS diseases confirmed by pathological diagnosis in Tibet,among which non-neoplastic diseases account for 1/3 of all the cases.Infectious and vascular diseases are the most common non-neoplastic diseases in Tibet,and tuberculosis and parasitic infections are relatively common.The types and proportion of brain tumors in Tibet are different from those in other regions of China,and meningioma is the most common in Tibet,with higher proportion than neuroepithelial tumor.

Microscopic resection of lumbar intraspinal tumor through keyhole approach: A clinical study of 54 cases / 北京大学学报(医学版)

OBJECTIVE@#To explore the feasibility and key technology of microscopic resection of lumbar intraspinal tumor through microchannel keyhole approach.@*METHODS@#The clinical features, imaging characteristics and surgical methods of 54 cases of lumbar intraspinal tumor which were microscopically operated by microchannel from February 2017 to September 2019 were reviewed and analyzed. There were 8 cases of extradural tumor, 3 cases of extra-and intradural tumor and 43 cases of subdural extramedullary tumor (including 3 cases of ventral spinal tumor). The tumors were 0.5-3.0 cm in diameter. The clinical symptoms included 49 cases of pain in the corresponding innervation area, 5 cases of sensory disturbance (numbness) at or below the tumor segment, 7 cases of limb weakness and 2 cases of urination and defecation dysfunction.@*RESULTS@#In the study, 37 tumors were resected through hemilaminectomy, 14 tumors were resected through interlaminar fenestration, 3 tumors were resected through hemilaminectomy or interlaminar fenestration combined with facetectomy of medial 1/4 facet. All of the 54 tumors were totally resected. The operation time was 75-135 min, with an average of 93.3 min. The postoperative hospital stay was 4-7 days, with an average of 5.7 days. Postoperative pathology included 34 cases of schwannoma, 4 cases of meningioma, 9 cases of ependymoma, 1 case of enterogenous cyst, 5 cases of teratoma/epidermoid/dermoid cyst, and 1 case of paraganglioma. No infection or cerebrospinal fluid leakage was found after operation. No neurological dysfunction occurred except 1 case of urination dysfunction and 4 cases of limb numbness. The follow-up period ranged from 3 to 33 months with an average of 14.4 months. Five patients with new onset symptoms returned to normal. The pain symptoms of 49 patients were completely relieved; 4 of 5 patients with hypoesthesia recovered completely, the other 1 patient had residual mild hypoesthesia; 7 patients with limb weakness, and 2 patients with urination and defecation dysfunction recovered to normal. No spinal instability or deformity was found, and no recurrence or residual tumors were found. According to McCormick classification, they were of all grade Ⅰ.@*CONCLUSION@#The lumbar intraspinal extramedullary tumors within two segments (including the ventral spinal tumors) can be totally resected at stage Ⅰ through microchannel keyhole approach with appropriate selection of the cases. Microchannel technique is beneficial to preserve the normal structure and muscle attachment of lumbar spine, and to maintain the integrity and stability of lumbar spine.

Rare Case of Young Patient with Intraventricular Angiomatous Meningioma

Pediatricmeningiomas are rare and account for only 2.2% of the central nervous system (CNS) tumors. In this age group, they aremore frequently located in atypical sites, such as, mainly, the ventricular system, with a frequency of 8.8 to 13.6%. Adding this to the fact that the angiomatous subtype constitutes only 2.1% of allmeningiomas, the rarity of the case reported here is corroborated. We report a 17-year-old female patient diagnosed with intraventricular angiomatous meningioma; she underwent surgical resection of the tumor in the body and frontal horn of the right lateral ventricle, and there were no neurological sequelae. With a follow-up of 26 months, there was no recurrence and the patient had clinical stability. Intraventricular tumors usually have slow growth and reach a considerable size until they cause symptoms and then are diagnosed. In addition, the deep location of the tumor and its proximity to eloquent areas make these tumors a neurosurgical challenge. The angiomatous subtype, due to the presence of hypervascularization (consisting of > 50% of vascular components), may, in some cases, hinder surgical resection as well as be erroneously diagnosed. However, surgical treatment aimed at total resection of the lesion remains the conduct of choice in the case reported here, especially in patients in the first two decades of life, in which the use of radiation is avoided. Specifically when it comes to the surgery, we chose a transcalosal approach that allows a good transoperative visualization of the lesion when located in the body and frontal horn of the lateral ventricle.

Sarcoidosis Mimicking Skull Base Meningioma

Sarcoidosis is a systemic disease characterized by granulomatous inflammation. Pulmonary and lymphatic granulomatous involvement are common.We present a rare case report of involvement of the central nervous system affecting the ocular region and mimicking optic nerve sheath meningioma. We report the case of a 79-year-old female patient with progressive visual impairment with an evolution of 4 years. Amagnetic resonance imaging scan of the cranium with gadolinium and intense homogeneous contrast enhancement revealed an expansive lesion in the right optic nerve, at the height of the optic canal. The patient was submitted to the neurosurgical approach with lesion biopsy, which showed sarcoidosis of the central nervous system. Due to the rarity of central nervous system involvement, the diagnosis of this pathologymay unfortunately be postponed. The present article aims to elucidate this pathology as a differential diagnosis of retro-orbital tumors.

A new approach for leptomeningeal metastases: chemotherapy administered through lumbar intrathecal port / Un nuevo abordaje para el tratamiento de las metástasis leptomeníngeas: uso de catéter implantado para el suministro de quimioterapia vía intratecal lumbar

ABSTRACT Background: Intrathecal chemotherapy is a local therapeutic modality used for treatment of leptomeningeal metastases. However, the techniques currently used, i.e. repeated lumbar puncture and Ommaya reservoir, have certain disadvantages. Lumbar intrathecal port (LIP) placement is a relatively novel technique, which has been used for pain management in cancer patients. Objective: To investigate the use of LIP for intrathecal administration of chemotherapeutic agents in patients with leptomeningeal metastases. Methods: Retrospective study of 13 patients treated with intrathecal chemotherapy for secondary leptomeningeal involvement of a primary solid tumor were included in this retrospective study. The patients received intrathecal chemotherapy through a LIP. Results: The patients received a total of 123 intrathecal chemotherapy doses. No grade 3-4 toxicity, technical problem or severe complication developed. During a median of 136 days of follow-up (range, 67-376 days), 12 patients died (92.3%). The treatment resulted in symptom improvement in all patients and self-rated overall health and quality of life improved, compared with baseline. Conclusions: The LIP system, which has been used for intrathecal pain management for decades, appears to offer a safe alternative for intrathecal chemotherapy in patients with leptomeningeal metastases. Further studies are warranted to clarify its potential use in this setting.

RESUMEN Antecedentes: La quimioterapia intratecal es una modalidad terapéutica local utilizada para el tratamiento de metástasis leptomeníngeas. Sin embargo, las técnicas empleadas actualmente, es decir, las punciones lumbares repetidas y el depósito de Ommaya, tienen algunos inconvenientes. La colocación de un puerto intratecal lumbar (LIP) es una técnica relativamente nueva que se ha utilizado para el tratamiento del dolor en pacientes con cáncer. Objetivo: Investigar el uso de LIP para la administración intratecal de agentes quimioterapéuticos en pacientes con metástasis leptomeníngeas. Métodos: Este estudio retrospectivo incluyó un total de 13 pacientes tratados con quimioterapia intratecal por afectación leptomeníngea secundaria de un tumor sólido primario. Los pacientes recibieron quimioterapia intratecal a través de un LIP. Resultados: Los pacientes recibieron un total de 123 dosis de quimioterapia intratecal. No se desarrolló toxicidad de grado 3-4, ni se presentaron problemas técnicos o complicaciones graves. Durante un promedio de 136 días de seguimiento (rango, 67-376 días), murieron 12 pacientes (92,3 %). El tratamiento dio como resultado una mejoría de los síntomas en todos los pacientes. La salud general autoevaluada y la calidad de vida mejoraron en comparación con los valores iniciales. Conclusiones: El sistema LIP que se ha utilizado para el manejo del dolor intratecal durante décadas, parece ofrecer una alternativa segura para la quimioterapia intratecal en pacientes con metástasis leptomeníngeas. Serán necesarios más estudios para determinar su uso potencial en este ámbito.

Epidemiological features of meningiomas: a single Brazilian center's experience with 993 cases / Características epidemiológicas de meningiomas: experiência de um centro único brasileiro com 993 casos

ABSTRACT Background: Meningiomas are the most frequent primary central nervous system (CNS) tumors. Their geographical and ethnic characteristics need to be known, in order to enable rational treatment. Objective: To investigate clinical and epidemiological aspects in a series of patients with meningiomas. Methods: Retrospective analysis on the demographic profile, location and histopathology of 993 patients with meningiomas (768 operated and 225 not operated). Results: Meningiomas represented 43.8% of the primary CNS tumors; 6.8% were multiple tumors (14.7% with neurofibromatosis 2) and 0.6% were radiation-induced tumors. The mean ages were 53.0 and 63.9 years for operated and non-operated patients and the female/male ratios were 3.2:1 and 6.3:1. Diagnosis was made later among females. The peak incidences were in the 6th and 7th decades respectively for operated and non-operated patients. The incidence was low at early ages and higher among patients aged 70+ years. The meningiomas were intracranial in 96.5% and most were WHO grade I (88.9%) and transitional. In the spinal canal (3.5%), they occurred mainly in the dorsal region (all grade I; mostly transitional). The racial distribution was 1.0% in Asian-Brazilians, 87% in Caucasians and 12% in African-Brazilians. 83.4% and 51.6% of the patients were estimated to be recurrence-free at 10 and 20 years, and the mortality rate was 3%. Conclusions: Most of the demographic data were similar to what has been observed in other western centers. Differences were higher incidence of meningiomas, female and older predominance in non-operated patients, predominance in Caucasian, and higher association with neurofibromatosis 2.

RESUMO Antecedentes: Meningiomas são os tumores mais frequentes do sistema nervoso central (SNC). Suas características étnicas e geográficas precisam ser conhecidas para o seu tratamento racional. Objetivo: Investigar aspectos clínicos e epidemiológicos de uma série de pacientes com meningiomas. Métodos: Análise retrospectiva demográfica de 993 pacientes com meningiomas (768 operados e 225 tratados conservadoramente) Resultados: Meningiomas constituíram 43.8% dos tumores primários do SNC. 0.8% deles eram múltiplos (14,7% com neurofibromatose 2) e 0,6% eram radioinduzidos. A idade média e o índice mulheres/homens foram respectivamente 53,0 e 63,9 anos e 3.2:1 e 6.3:1 para pacientes operados e não operados. O diagnóstico foi mais tardio em mulheres. Ocorreram picos de incidências na 6ª e na 7ª décadas respectivamente para pacientes operados e não operados. A incidência foi menor na infância e maior após 70 anos. Meningiomas predominaram no crânio (96.5%), a maioria grau I da OMS, subtipo transicional. Do total, 3.5% ocorreram no canal raquídeo, principalmente na região torácica, todos grau I, a maioria transicional. Em relação à distribuição racial, 1.0% dos meningiomas ocorreu em amarelos, 87% em brancos e 12% em negros. As taxas de sobrevida sem recorrência foram 83.4% e 51.6% em 10 e 20 anos e a mortalidade operatória foi 3%. Conclusões: A maioria dos dados demográficos observados foi similar aos de outros centros ocidentais. As diferenças observadas foram maior incidência, predominância em mulheres e idosos nos pacientes não operados e em caucasianos, e maior associação com neurofibromatose 2.

Extracerebral Pterional Metastasis from Malignant Meningioma: A Case Report

Anaplastic or malignant meningiomas that are classified as World Health Organization (WHO) grade III account for less than 1% of all meningiomas diagnosed. Despite the aggressive course, distant metastases are a rarity, occurring in only 0.1% to 0.2% of cases. We report the case of a 56-year-old woman who presented with parasagittal benign meningioma that underwent malignant transformation along with metastasis into the right orbitosphenoid region. The clinical, radiological, and histopathological aspects are highlighted, with an emphasis on the natural history of meningiomas.

Psicosis secundaria a meningioma: revisión a propósito de un caso / Secondary psychosis due to meningioma: review about a case

Resumen Los meningiomas son los tumores primarios más frecuentes del sistema nervioso central, tienden a ser benignos y de lento crecimiento. Pueden ser asintomáticos o incluso manifestarse únicamente con síntomas psiquiátricos, incluyendo un cuadro psicótico. No existen estudios clínicos controlados randomizados que estudien la relación entre meningioma y cuadros psicóticos. La evidencia disponible se basa en series y reportes de casos. Existe una relación entre la magnitud del edema perilesional y la presencia de síntomas psicóticos. Por otra parte, el tamaño de la lesión o su localización neuroanatómica específica tendrían menor relevancia. La resección quirúrgica de la lesión, en conjunto con el manejo psiquiátrico adecuado, usualmente conduce al cese de la sintomatología psicótica. En la evaluación de pacientes con síntomas psicóticos se debe tener un elevado índice de sospecha, en particular en cuadros de reciente inicio, con manifestaciones atípicas o resistentes al tratamiento. En estos casos se recomienda un estudio con neuroimágenes. Este artículo presenta el caso de una paciente evaluada en nuestro hospital diagnosticada con un meningioma frontal izquierdo de gran tamaño, que presentó sintomatología psicótica secundaria, y se expone una revisión bibliográfica actualizada de esta asociación.

Meningiomas are the most frequent central nervous primary tumors, which tend to be benign and present a slow growth. They may be asymptomatic or present clinically just with psychiatric symptoms including a psychotic state. There are no clinical randomized controlled trials that study the relationship between meningioma and a psychotic episode. Available evidence is based on case reports and series. There is a relationship between the magnitude of perilesional edema and the presence of psychotic symptoms. On the other hand, the size of the tumor or its specific neuroanatomic location would have less relevance. Surgical resection of the tumor associated with psychiatric management usually leads to the cessation of psychotic symptoms. In the assessment of patients with psychotic symptoms, there must be a high index of suspicion, particularly in first psychotic episodes, atypical manifestations and resistance to treatment. In these cases, a neuroimaging study is recommended. This article presents the case of a patient evaluated in our hospital and diagnosed with a large left frontal meningioma with secondary psychotic symptoms, and an updated bibliographic review of this association is presented.

Analysis on endoscopic endonasal approach combined with the frontotemporal orbitozygomatic approach under the microscope for skull base tumor with intra and extra-cranial involvement / 中华耳鼻咽喉头颈外科杂志

Objective: To evaluate the application of combination use of endoscopic endonasal approach combined with the frontotemporal orbitozygomatic approach microscopically in skull base tumor with intra and extra-cranial involvement. Methods: A total of 7 patients (4 males and 3 females, aging from 27 to 65 years old, with a medium age of 48) undergone complicated skull base surgeries via endoscopic endonasal approach combined with the frontotemporal orbitozygomatic approach microscopically from May 2016 to January 2018 were reviewed respectively. The patients included 2 cases of recurrent invasive pituitary adenoma, 3 cases of basal skull meningiomas, 1 case of clivus chondrosarcoma, and 1 case of recurrent nasopharyngeal carcinoma. The lesion extensively infiltrated nasal cavity, extending to the paranasal sinus, bilateral cavernous sinus, sellar region, suprasellar, superior clivus, temporal lobe, pterygopalatine fossa, infratemporal fossa and important intracranial vessels. All the 7 patients were treated under general anesthesia by endoscopic endonasal approach combined with frontotemporal orbitozygomatic approach under the microscope. Total excision rate, intraoperative and postoperative complications and postoperative curative effect were observed. All of them were followed up for 6 to 12 months. The Glasgow Outcome Scale (GOS) was used to evaluate the prognosis. Result: Total tumor removal was performed in 5 cases, subtotal removel in 2 cases. There was no complication during the operation. Postoperative severe complications occurred in 2 cases, including 1 case of cerebrospinal fluid rhinorrhea and intracranial infection, which was cured by lumbar cistern drainage and intrathecal injection; 1 case occurred oculomotor nerve paralysis, which recovered during follow-up. Postoperative complications occurred in 1 case of trochlear nerve dysfunction, 2 cases of facial numbness, and 1 case of tinnitus. During follow-up, all patients recovered to varying degrees. There was no bleeding and death after the operation. No tumor recurred during the follow-up period. All patients were recovered well with GOS grade Ⅳ-Ⅴ. Conclusions: Endoscopic transnasal approach combined with microscopic frontotemporal orbitozygomatic approach can remove tumors in one stage, reduce surgical complications and improve surgical effect. It has good application prospects and is suitable for excising complex intracranial and extracranial communicating tumors of widely involving sellar, clivus and petrous apex area.

Intracranial Inflammatory Myofibroblastic Tumor: A Review of 49 cases

Inflammatory Myofibroblastic Tumor (IMT) is a rare pathologic entity that was first described in 1973. This lesion is most commonly found in the lungs, but other organs' involvement has also been reported. Intracranial location of Inflammatory Myofibroblastic Tumor is rare, and the first case was reported in 1980. An intriguing fact about the intracranial IMT is its resemblance with meningioma on clinical presentation and neuroimaging. We came across a case of intracranial Inflammatory Myofibroblastic Tumor (IIMT) in a 27-year-old male who presented with recurrent episodes of seizures and was diagnosed as meningioma on neuroimaging. The lesion did not subside with medical management and kept on progressing in size. The patient had to undergo surgery, and diagnosis of Inflammatory Myofibroblastic Tumor was ascertained on histopathology. This 'surprise' diagnosis prompted us to review the literature on all cases of IIMTs reported to date to better understand the entity and its implications. In this review article, we present our observations regarding various studied parameters, including patient profile, clinical presentation, site of involvement, focality of the lesion, special associations, and lines of management of the 49 published cases of IIMTs.

Magnetic Resonance Imaging for Diagnosis of Idiopathic Pediatric Meningiomas / 中国医学科学院学报

Objective To analyze the radiological features of idiopathic pediatric meningiomas and explore their relationships with pathological grading,misdiagnoses,and blood loss during surgery.Methods We retrospectively reviewed 29 cases of pathologically confirmed pediatric meningiomas with pre-operative magnetic resonance imaging in Beijing Tiantan Hospital from November 2014 to July 2018.We assessed the imaging features to explore their relationships with pathological grading,misdiagnoses,and blood loss during surgery. Results Among the 29 cases,7 intraparenchymal meningiomas,5 extraparenchymal meningiomas,4 ventricular meningiomas,and 1 transcranial meningioma were misdiagnosed.Tumor location was significantly associated with possibility of misdiagnoses(

El análisis inmunohistoquímico para el diagnóstico del tumor fibroso solitario/hemangiopericitoma / Immunohistochemical analysis for the diagnosis of solitary fibrous tumor/hemangiopericytoma

Resumen El tumor fibroso solitario/ hemangiopericitoma (TFS/HP) es un tumor extraaxial de origen mesenquimático de infrecuente observación, que usualmente se confunde con el meningioma, del cual puede ser clínica y radiológicamente indistinguible. El análisis molecular con la detección de la expresión nuclear STAT6 (signal transducer and activator of transcription 6) o la fusión NAB2-STAT6 (NGFI-A binding protein 2) es recomendable para confirmar el diagnóstico. Presentamos 3 casos clínicos, 2 mujeres y 1 varón, con diagnóstico anatomopatológico de meningioma meningotelial en el primer caso; y los casos 2 y 3 con sospecha radiológica de meningioma. La revisión anatomopatológica con estudio molecular permitió certificar el diagnóstico de TFS/ HP. Para el diagnóstico diferencial entre TFS/HP meníngeo y meningioma, se recomienda buscar la expresión de STAT6 como primer paso o la fusión NAB2-STAT6. La revisión de las muestras de biopsia debe estar garantizada en todos los pacientes, inclusive en aquellas que fueron estudiadas en Servicios de Patología Nivel 3.

Abstract The solitary fibrous tumor/ hemangiopericytoma (TFS/HP) is a rare mesenchymal extraaxial tumour. TFS/HP can sometimes be difficult to distinguish from other extra-axial tumors like meningioma, which can be clinically and radiologically indistinguishable. Molecular analysis with STAT6 (signal transducer and activator of transcription 6) nuclear expression or NAB2-STAT6 (NGFI-A binding protein 2) fusion is recommended to confirm the diagnosis. We present 3 cases, 2 women and 1 male, with pathological diagnosis of meningothelial meningioma in the first case; cases 2 and 3 with radiological suspicion of meningioma. The pathological review with molecular study certified the diagnosis of TFS/HP. For differential diagnosis between meningeal TFS/HP and meningioma, it is recommended to look for STAT6 expression as a first step, or NAB2-STAT6 fusion in order to confirm TFS/HP. The review of biopsy samples must be guaranteed in all patients, including those who were studied in Pathology Services Level 3.

A Diffuse Leptomeningeal Glioneural Tumor Case Producing Hydrocephalus and Polyradiculopathy

The present report describes the case of a male 17-year-old patient who progressively developed a hydrocephalus and polyradiculopathy due to involvement of central nervous system (CNS) by a diffuse leptomeningeal glioneuronal tumor (DLGNT). The tumor had partial remission in response to the treatment with radiotherapy plus procarbazine, lomustine, and vincristine (PCV) chemotherapy, and the patient had improvement in function and pain levels. The current knowledge about DLGNT, including its clinical manifestations, imaging findings, histological characteristics, and treatment are revised and discussed in the present paper.

Melanocitoma meníngeo del ángulo pontocerebeloso: claves imagenológicas para su abordaje / Cerebellopontine meningeal melanocytoma: keys for imaging diagnostic approach

Resumen: Las lesiones del ángulo pontocerebeloso (APC) representan el 6 al 10% de las neoplasias intracraneales, siendo los schwannomas vestibulares y meningiomas los más comunes. Sin embargo, hasta el 15% pueden ser otras lesiones, entre ellas las derivadas a partir de restos de células melanocíticas presentes en las leptomeninges. El diagnóstico diferencial de las patologías tumorales del APC es extenso, siempre teniendo en cuenta las lesiones más comunes. Sin embargo, cuando las características radiológicas no son las esperadas, el enfoque debe orientarse hacia las lesiones inusuales, poniendo en contexto las diferentes estirpes celulares que pueden dar origen a las neoplasias en esta localización, como las neoplasias melanocíticas. Se presenta el caso de un masculino de 74 años con síndrome cerebeloso de tórpida evolución, al cual se le realiza RM de cerebro contrastada, identificando una lesión de base dural en el APC izquierdo, con hiperintensidad de señal en T1 e hipointensidad en T2, atípico para las lesiones más comunes en esta región, que sugiere su contenido melanocítico.

Abstract: Cerebellopontine angle tumors (CPA) represent approximately 6 to 10% of intracranial tumors. Vestibular Schwannomas and meningiomas are the most common, however up to 15% can be of other origin, including from melanocytes derived from the neural crest. The differential diagnosis of CPA pathologies is extensive, always taking into account the most common ones. However, if the radiological characteristics are not the expected, the approach should be directed towards unusual lesions, putting into context the different cell lines that can give rise to the neoplasm at this location, such as melanotic neoplasms. We present a case of a 74-year-old male, who presented with a cerebellar syndrome. Due to an atypical clinical evolution, a contrast enhanced head MRI was performed, revealing a dural based tumor on the left CPA, which was hyperintense on T1 and hypointense on T2 weighted sequences, which is not expected from the common lesions at this region and suggested it's melanotic content.

Lymphoplasmacyte-Rich Meningioma of the Third Ventricle: Case Report

Introduction Meningiomas are the most common primary intracranial tumors, accounting for up to 35% of the neoplasms in this category. Approximately 10­20% of these neoplasms are histologically atypical, and the lymphoplasmacyte-rich meningioma (LPM) corresponds to a very rare subtype of meningioma that is characterized histopathologically by massive infiltrates of inflammatory cells. The case described in the present study is the sixth case of an intraventricular LPM found in the literature and the first case considering the location in the third ventricle. Case Description A 21-year-old male without previous comorbidities sought medical attention due to visual impairment (complaining of intermittent visual blur) for 2 months. A magnetic resonance imaging of the brain confirmed the presence of a well-delimited solid mass in the third ventricle of 3.0 2.3 cmwith a cystic component that extended itself inferiorly and distorted the visual pathway anatomy. Neurosurgeons decided to access the lesion using an interhemispheric transcallosal approach with a transforaminal access, and the lesion was resected completely. The patient has an ambulatorial endocrinological follow-up and is neurologically stable 6 months after the procedure. No new visual deficits were noted. Conclusion Lymphoplasmacyte-rich meningioma is a very rare intracranial tumor, and the involvement of the third ventricle make this case unique.

Tailored Surgical Approaches for Foramem Magnum Tumors

Objective To describe our surgical techniques, analyze their safety and their postoperative outcomes for foramen magnum tumors (FMTs). Methods From 1986 to 2014, 34 patients with FMTs underwent surgeries using either the lateral suboccipital approach, standard midline suboccipital craniotomy, or the far lateral approach, depending on the anatomic location of the lesions. Results In the present series, there were 22 (64.7%) female and 12 (35.2%)male patients. The age of the patients ranged from12 to 63 years old.We observed 1 operativemortality (2.9%). A total of 28 patients (82.3%) achieved a score of 4 or 5 in the Glasgow Outcome Scale (GOS). Gross total resection (GTR) was obtained in 22 (64.7%) patients. After the surgery, 9 (26%) patients developed lower cranial nerve dysfunction (LCNd) weakness. The follow-up varied from 1 to 24 years (mean: 13.2 years). Conclusion Themajority of tumors located in the FMcan be safely and efficiently removed usingeither thelateral suboccipital approach, standardmiddlelinesuboccipital craniotomy, or the far lateral approach, depending on the anatomic location of the lesions.