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.

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.

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.

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.

Endoscopic endonasal double flap technique for reconstruction of large anterior skull base defects: technical note / Técnica endoscópica endonasal utilizando duplo retalho para reconstrução de grandes defeitos da base anterior do crânio: nota técnica

Abstract Introduction: One of the main concerns in endoscopic endonasal approaches to the skull base has been the high incidence and morbidity associated with cerebrospinal fluid leaks. The introduction and routine use of vascularized flaps allowed a marked decrease in this complication followed by a great expansion in the indications and techniques used in endoscopic endonasal approaches, extending to defects from huge tumours and previously inaccessible areas of the skull base. Objective: Describe the technique of performing endoscopic double flap multi-layered reconstruction of the anterior skull base without craniotomy. Methods: Step by step description of the endoscopic double flap technique (nasoseptal and pericranial vascularized flaps and fascia lata free graft) as used and illustrated in two patients with an olfactory groove meningioma who underwent an endoscopic approach. Results: Both patients achieved a gross total resection: subsequent reconstruction of the anterior skull base was performed with the nasoseptal and pericranial flaps onlay and a fascia lata free graft inlay. Both patients showed an excellent recovery, no signs of cerebrospinal fluid leak, meningitis, flap necrosis, chronic meningeal or sinonasal inflammation or cerebral herniation having developed. Conclusion: This endoscopic double flap technique we have described is a viable, versatile and safe option for anterior skull base reconstructions, decreasing the incidence of complications in endoscopic endonasal approaches.

Resumo Introdução: Uma das principais preocupações em abordagens endoscópicas endonasais da base do crânio tem sido a alta incidência e morbidade associada a fístulas liquóricas. A introdução e o uso rotineiro de retalhos vascularizados permitiram uma acentuada redução dessa complicação, seguida por uma grande expansão nas indicações e técnicas utilizadas nas abordagens endoscópicas endonasais, incluindo grandes tumores e áreas anteriormente inacessíveis da base do crânio. Objetivo: Descrever a técnica cirúrgica realizando uma reconstrução endoscópica multicamadas da base anterior do crânio com duplo retalho, sem craniotomia. Método: Descrição passo a passo da técnica endoscópica com duplo retalho (retalhos vascularizados nasoseptal e pericraniano e enxerto livre de fascia lata), utilizados e ilustrados em dois pacientes com meningioma do sulco olfatório submetidos à cirurgia por via endoscópica endonasal. Resultados: Em ambos os pacientes procedeu-se ressecção total macroscópica seguido de reconstrução da base anterior do crânio com os retalhos nasoseptal e pericraniano onlay e enxerto livre de fáscia lata inlay. Os pacientes apresentaram uma excelente recuperação, sem sinais de fístula liquórica, meningite, necrose do retalho, inflamação meníngea crônica ou sinonasal ou hérnia cerebral. Conclusão: A técnica endoscópica de duplo retalho, como descrita, trata-se de uma opção viável, versátil e segura para as reconstruções da base anterior do crânio, diminuindo a incidência de complicações em abordagens cirúrgicas endoscópicas endonasais.

Una imagen atípica para un tumor típico / An atypical imaging for the most typical intracranial tumor

Los meningiomas son los tumores intracraneales benignos más frecuentes en adultos, suponen el 20% de todos los tumores cerebrales. Solamente el 1-4% presentan cambios quísticos. La relación entre el componente quístico y sólido de los meningiomas mixtos fue utilizada por varios autores para crear hasta cuatro clasificaciones diferentes de este subtipo de meningiomas. El diagnóstico mediante TC resulta muy complejo ante este subtipo de meningiomas por la dificultad de discernir entre diferentes lesiones intracraneales que también presentan componente quístico asociado. Presentamos el caso de un paciente varón de 49 años que presenta episodios de amaurosis fugax. En la TC se identificó una lesión sólida en localización frontotemporal izquierda, con gran componente quístico que producía importante efecto de masa y desplazaba la línea media. Se completó estudio mediante RM y arteriografía y finalmente fue diagnosticado como meningioma mixto, que tras embolización tumoral desde arteria meníngea media, se resecó quirúrgicamente.

Meningiomas are the most common benign intracranial tumors in adults, accounting for 20% of all brain tumors. Only 1-4% have cystic changes. The relationship between the cystic and solid component of mixed meningiomas was used by several authors to create up to four different classifications of this subtype of meningiomas. CT diagnosis is challenging in this subtype of meningiomas because of the difficulty of distinguishing between different intracranial lesions that also have associated cystic component. We present the case of a 49-year-old male patient with episodes of amaurosis fugax. On CT, a solid lesion was identified in the left frontotemporal region, with a large cystic component that produced an important mass effect and midline displacement. A study was completed by MR and arteriography and finally was diagnosed as mixed meningioma, which after tumor embolization via the middle meningeal artery, was surgically resected.

Simultaneous meningioma and brain metastasis from renal cell carcinoma - a rare presentation. Case report / Meningioma e metástase cerebral de carcinoma de células renais simultâneos - uma apresentação rara. Relato de caso

ABSTRACT CONTEXT: Brain metastases are the most common tumors of the central nervous system. Because of their high frequency, they may be associated with rare situations. Among these are tumor-to-tumor metastasis and an even a rarer situation called simultaneous brain tumors, which are more related to primary tumors of the reproductive and endocrine systems. CASE REPORT: A 56-year-old male patient with a history of renal cell carcinoma (which had previously been resected) presented with a ventricular lesion (suggestive of metastatic origin) and simultaneous olfactory groove lesion (probably a meningioma). First, only the ventricular lesion was dealt with, but after a year, the meningothelial lesion increased and an occipital lesion appeared. Therefore, both of these were resected in a single operation. All the procedures were performed by the same neurosurgeon. The patient evolved without neurological deficits during the postoperative period. After these two interventions, the patient remained well and was referred for adjuvant treatment. CONCLUSIONS: This study provides the first description of an association between these two tumors. Brain metastases may be associated with several lesions, and rare presentations such as simultaneity with meningioma should alert neurosurgeons to provide the best oncological treatment.

RESUMO CONTEXTO: As metástases cerebrais são os tumores mais comuns do sistema nervoso central e, devido à sua elevada frequência, podem estar associadas a situações raras. Entre estas estão as "tumor to tumor metastasis" e uma situação ainda mais rara chamada de tumores cerebrais simultâneos, mais relacionados a tumores primários dos sistemas endocrinológico e reprodutivo. RELATO DE CASO: Um homem de 56 anos com histórico de câncer de células renais (extirpado previamente) apresentou-se com lesão ventricular (sugestiva de origem metastática) e simultaneamente com uma lesão em topografia de goteira olfatória (provavelmente meningioma). Primeiramente, apenas a lesão ventricular foi abordada, porém após um ano, a lesão meningotelial aumentou e uma lesão occipital apareceu e então ambas foram ressecadas em uma única cirurgia. Todos os procedimentos foram realizados pelo mesmo neurocirurgião. O paciente evoluiu sem déficits neurológicos no período pós-operatório. Após essas duas intervenções, o paciente permaneceu bem, sendo encaminhado para tratamento adjuvante. CONCLUSÕES: O presente trabalho é a primeira descrição da associação encontrada entre esses dois tumores. As metástases cerebrais podem associar-se a várias lesões, e manifestações raras, tais como apresentação simultânea com meningioma, devem alertar o neurocirurgião a fornecer o melhor tratamento oncológico.

Metástasis durales: una forma infrecuente de recurrencia en tumores nasosinusales malignos / Dural metastasis: an uncommon form of recurrence in malignant sinonasal tumours

Abstract: Dural metastases are an unusual form of spread in treated sinonasal malignancies. An analysis is presented of 20 cases of dural metastases diagnosed during imaging follow-up in a selection of cases in which anterior craniofacial resection was performed. They included 12 undifferentiated sinonasal carcinomas, 7 olfactory neuroblastomas, and 1 adenoid cystic carcinoma case. Dural metastases appeared on an average of 7.3 years after treatment in olfactory neuroblastoma. The maximum distance from malignancy to dural metastases was 14 cm for olfactory neuroblastoma, and 4.3 cm for undifferentiated sinonasal carcinoma. Dural metastases in the Burr holes were observed in 50% of undifferentiated sinonasal carcinoma, and 29% of olfactory neuroblastomas. Dural metastases presented as a nodular (60%), multinodular (10%), cystic (15%), and plaque (15%) pattern. These are suggestive of a local venous spread mechanism related to tumour rupture during surgery of anterior cranial fossa. Long-term follow-up with cranial inclusion would be indicated, given the possible late and distant presentation of dural metastases.

Resumen: Presentamos las metástasis durales como forma inusual de diseminación de tumores nasosinusales malignos tratados; se revisan 20 casos diagnosticados durante el seguimiento imagenológico a un grupo tratado con resección craneofacial anterior. Evaluamos metástasis durales en 12 carcinomas nasosinusales indiferenciados, 7 neuroblastomas olfatorios y un carcinoma adenoquístico. En neuroblastomas olfatorios aparecieron metástasis durales en promedio 7,3 años postratamiento. La distancia máxima del tumor a la metástasis fue de 14 cm para neuroblastoma olfatorio y de 4,3 cm para carcinoma nasosinusal indiferenciado. Observamos metástasis durales en los agujeros de trepanación en el 50% de los carcinomas nasosinusales indiferenciados y en el 29% de los neuroblastomas olfatorios. Las metástasis durales presentaron patrón nodular (60%), multinodular (10%), quístico (15%) y en placa (15%). Proponemos un mecanismo venoso local de diseminación relacionado a disrupción tumoral o quirúrgica de la fosa craneal anterior. El seguimiento a largo plazo con inclusión craneal estaría indicado por la posible presentación tardía y distante de metástasis durales.

Extreme lateral transcondylar approach to the skull base.

In this study, the authors present their experience of using extreme later transcondylar approach (ELTC) for treating 7 patients with lesions in the anterolateral foramen magnum, upper cervical spine and cerebellopontine angle reaching upto jugular foramen. The tumours included meningiomas, neurofibromas (2 cases each), chondrosarcoma, epidermoid and aneurysmal bone cyst (one case each). The approach was used alone, in combination with retrolabyrinthine presigmoid approach in a patient with lower cranial nerve neurofibroma extending extracranially through the jugular foramen, or in combination with partial C1-C3 laminectomy in two patients with meningiomas situated anterolateral to the cord from the foramen magnum to C3. In two patients with extradural vertebral artery (VA) entrapment by a chondrosarcoma and aneurysmal bone cyst respectively, the vertebral artery was ligated distal to the tumour. The tumours were totally excised in five cases and partially in two. There was no preoperative mortality. The major complications included cerebrospinal fluid leak from the wound (3 cases) and increase in lower cranial nerve paresis (2 cases). At follow up, ranging from 6 months to 2 years, 5 patients showed no tumour recurrence. There was improvement in neurological status. One patient, with a partially excised aneurysmal bone cyst, showed no added deficits or increase in the tumour size. However, there was a massive regrowth in the patient with chondrosarcoma after 6 months. This technique provided a wide surgical exposure with direct visualization of the tumour-anterior cord interface, early proximal control of the VA and preservation of lower cranial nerves.

Hypodense meningiomas.

While majority of the meningiomas can be diagnosed on contrast enhanced computerised tomography (CT) scans, few of them can have atypical appearances, which may be difficult to distinguish from those seen in glial tumours. Six atypical cases of meningioma having hypodense appearances on CT scan are described.

CT findings of atypical meningiomas including aggressive and malignant forms

This study included 54 selected patients [42 females and 12 males with ages ranging between 28 and 63 years] examined by CT scan and plain X- ray for the skull. These cases showed CT findings of an intracranial neoplastic lesions not conformed with typical radiological findings of meningioma. Invasive meningiomas and meningosarcoma were part of this work. Operative and histological verification of the diagnosis of all these cases were obtained

CT findings of atypical meningiomas including aggressive and malignant forms

The study included 54 selected patients, 42 females and 12 males, ages ranging between 28 to 63 years examined by CT scan and plain X-ray for the skull. These cases showed CT findings of an intracranial neoplastic lesions not conforming with typical radiological findings of meningioma. Invasive meningiomas and meningosarcoma were part of this work. Operative and histological verification of the diagnosis of all these cases were obtained