Spinal Cord Subependymoma Surgery: A Multi-Institutional Experience

OBJECTIVE: A spinal cord subependymoma is an uncommon, indolent, benign spinal cord tumor. It is radiologically similar to a spinal cord ependymoma, but surgical findings and outcomes differ. Gross total resection of the tumor is not always feasible. The present study was done to determine the clinical, radiological and pathological characteristics of spinal cord subependymomas. METHODS: We retrospectively reviewed the medical records of ten spinal cord subependymoma patients (M : F=4 : 6; median 38 years; range, 21–77) from four institutions. RESULTS: The most common symptoms were sensory changes and/or pain in eight patients, followed by motor weakness in six. The median duration of symptoms was 9.5 months. Preoperative radiological diagnosis was ependymoma in seven and astrocytoma in three. The tumors were located eccentrically in six and were not enhanced in six. Gross total resection of the tumor was achieved in five patients, whereas subtotal or partial resection was inevitable in the other five patients due to a poor dissection plane. Adjuvant radiotherapy was performed in two patients. Neurological deterioration occurred in two patients; transient weakness in one after subtotal resection and permanent weakness after gross total resection in the other. Recurrence or regrowth of the tumor was not observed during the median 31.5 months follow-up period (range, 8–89). CONCLUSION: Spinal cord subependymoma should be considered when the tumor is located eccentrically and is not dissected easily from the spinal cord. Considering the rather indolent nature of spinal cord subependymomas, subtotal removal without the risk of neurological deficit is another option.

Spinal Cord Subependymoma Surgery: A Multi-Institutional Experience

OBJECTIVE: A spinal cord subependymoma is an uncommon, indolent, benign spinal cord tumor. It is radiologically similar to a spinal cord ependymoma, but surgical findings and outcomes differ. Gross total resection of the tumor is not always feasible. The present study was done to determine the clinical, radiological and pathological characteristics of spinal cord subependymomas.METHODS: We retrospectively reviewed the medical records of ten spinal cord subependymoma patients (M : F=4 : 6; median 38 years; range, 21–77) from four institutions.RESULTS: The most common symptoms were sensory changes and/or pain in eight patients, followed by motor weakness in six. The median duration of symptoms was 9.5 months. Preoperative radiological diagnosis was ependymoma in seven and astrocytoma in three. The tumors were located eccentrically in six and were not enhanced in six. Gross total resection of the tumor was achieved in five patients, whereas subtotal or partial resection was inevitable in the other five patients due to a poor dissection plane. Adjuvant radiotherapy was performed in two patients. Neurological deterioration occurred in two patients; transient weakness in one after subtotal resection and permanent weakness after gross total resection in the other. Recurrence or regrowth of the tumor was not observed during the median 31.5 months follow-up period (range, 8–89).CONCLUSION: Spinal cord subependymoma should be considered when the tumor is located eccentrically and is not dissected easily from the spinal cord. Considering the rather indolent nature of spinal cord subependymomas, subtotal removal without the risk of neurological deficit is another option.

Tuberous sclerosis complex: Imaging characteristics in 11 cases and review of the literature / 华中科技大学学报（医学）（英德文版）

Tuberous sclerosis complex (TSC) is an uncommon multiorgan disorder that may present many and different manifestations on imaging. Radiology plays an important role in diagnosis and management, and can substantially improve the clinical outcome of TSC. Therefore, a comprehensive understanding of this disease is essential for the radiologist. The manifestations of TSC on computer tomography (CT) and magnetic resonance (MR) images were analyzed. Eleven patients with a clinical diagnosis of TSC were retrospectively reviewed. Central nervous system lesions included subependymal nodules (SENs) (11/11), subependymal giant cell astrocytomas (SEGAs) (2/11), cortical and subcortical tuber lesions (5/11), and white matter lesions (4/11). Of the 6 patients with abdominal scans, there were 6 cases of renal angiomyolipomas (AMLs), and one case of hepatic AMLs. Of the 4 patients undergoing chest CT, lung lymhangioleiomyomatosis (LAM) (2/4), and multiple small sclerotic bone lesions (2/4) were observed. Different modalities show different sensitivity to the lesion. Analysis of images should be integrated with patients' history in order to diagnose TSC.

Infratentorial and Intraparenchymal Subependymoma in the Cerebellum: Case Report

Subependymomas are rare benign tumors located in the ventricular system. Intraparenchymal subependymoma is extremely rare; only 6 cases have been reported, and all were located in the supratentorial region. We describe a case of infratentorial, intraparenchymal subependymoma in a 28-year-old man with intermittent headache. Imaging revealed a well-demarcated cystic and solid cerebellar mass near the fourth ventricle. The mass had a microcystic component and calcification without contrast enhancement. Complete surgical excision was performed, and histopathology confirmed a subependymoma.

Subependimoma sintomático del piso del IV ventrículo: reporte de un caso y revisión de la literatura / Simptomatic subependimoma of IV ventricle

Objetivo: reportar un caso de esta infrecuente patología y realizar una revisión bibliográfica, analizando sus características clínicas y morbimortalidad. Descripción: presentamos aquí el caso de un paciente de 64 años, el cual consulta sobre un síndrome vertiginoso de un año de evolución. En la IRM se observaba una lesión ocupante de espacio en el cuarto ventrículo, la cual media 5,3 x 4,1 x 4,2 cm. No se observaba franca hidrocefalia a pesar del tamaño de la misma. Intervención: se efectuó una craniectomía suboccipital con laminectomía C1. El tumor nacía francamente del piso del IV ventrículo. Se resecó la lesión en forma completa, efectuando cavitación y disección roma entre la cápsula y el piso IV ventrículo. El paciente tuvo un postoperatorio tórpido dada la paresia de pares bajos que presentó durante las primeras semanas, por lo que fue intubado, traqueostomizado y alimentado por sonda nasoyeyunal. Al 5º mes de postoperatorio el paciente se hallaba independiente para sus actividades de la vida diaria. La resonancia postoperatoria evidenciaba la exéresis completa de la lesión. Conclusión: los subependimomas que nacen del piso del IV ventrículo tienen una elevada morbimortalidad documentada. Sólo la minuciosa disección con microscopio y cuidados intensivos postoperatorios pueden mejorar este pronóstico...

Intracranial manifestations of tuberous sclerosis: a pictorial essay

Tuberous sclerosis is an autosomal dominant genetic disease that involves multiple organs. Hamartomas are the predominant lesions. Classically, tuberous sclerosis has been characterized by a classical clinical triad of facial angiofibromas [90%], mental retardation [50-80%], seizure [80-90%] and all three in 30% of the patients. Two major features or one major feature plus two minor features are necessary for the definite diagnosis of this disease. We had some patients admitted with different presentations of tuberous sclerosis and a past history of convulsion from childhood, skin lesions and also mental retardation with a new onset headache and a changed pattern of convulsion. In physical examination, facial angiofibromas and subungual fibromas were apparently detected. Brain CT scan study with contrast showed multiple calcified nodules associated with tubers, ventriculomegaly and also enhancing enlarged nodules at the foramen of Monro, which were suggestive of subependymal giant cell astrocytoma [SGCA]. MRI showed the same brain findings [tubers, white matter lesions and subependymal nodules associated with SGCA], which were detected better. After surgery, SGCA was proved. In abdominal and pelvic CT scan and ultrasonography, massive bilateral angiomyolipomatosis and focal hypodense hyperechoic liver lesions were detected

Intramedullary Subependymoma of the Thoracic Spinal Cord

An Intramedullary subependymoma of the spinal cord is a rare tumor with only 43 reported cases in the literature. Most of them are reported to be localized within the cervical spinal cord. We report a rare case of a thoracic spine intramedullary subependymoma in a 37-year-old female who presented with back pain and radiating leg pain. Subtotal resection and post-operative radiotherapy were performed. Subependymomas developing in the spinal cord are benign with a low proliferative potential. Complete resection of the tumor appears to be the optimal method for a complete cure. However aggressive surgery may cause severe neurological deficit. Therefore, if severe neurological deficits are expected after complete removal, a partial removal and postoperative radiation therapy is an alternative method for treatment in selected cases. A large-scale randomized study is mandatory to clarify the effectiveness of radiotherapy and to establish the recurrence rate and prognosis with respect to the surgical removal of these tumors.

Imágenes con tensor de difusión en resonacia magnética: aplicaciones clínicas / Diffusion Tensor MRI: clinical aplications

Propósito: Evaluar el comportamiento de diferentes entidades neurológicas utilizando imágenes ponderando tensor de difusión (ITD) y determinar si el método aporta información adicional a las imágenes de RM convencional. Material y método: Se estudiaron 8 pacientes portadores de distintas patologías encefálicas (5 casos de tumores, 1 paciente con esclerosis múltiple (EM), 1 paciente con enfermedad de Creutzfeldt-Jakob, variante esporádica (C-Je) y 1 paciente con intoxicación subaguda por monóxido de carbono (intoxicación por CO) en un equipo de RM de 1,5 T, utilizando secuencias convencionales e ITD con 25 direcciones. Se obtuvieron mapas cualitativos y se cuantificó la anisotropía fraccional (AF) ubicando diferentes regiones de interés según guías anatómicas específicas (cápsulas interna y externa, fibras frontocallosas y temporales, etc). Resultados: En los pacientes con tumores encefálicos, se observó disminución de AF sobre las fibras peritumorales, con distorción y/o disrupción de las mísmas. En los casos de EM, C-Je e intoxicación por CO, notamos interrupción parcial en el trayecto de los haces de sustancia blanca (SB) involucrados. Sin embargo, existió importante discordancia entre los hallazgos en FLAIR y Difusión (DWI) y en ITD, en el caso de intoxicación subaguda por CO. Conclusión: Las ITD, tanto cuali como cuantitativas aportan mayor información que las secuencias convencionales sobre la morfología ultraestructural del tejido cerebral en las patologías estudiadas. Además, estas imágenes constituyen la base de otra técnica de RM, actualmente en desarrollo, como lo es la Tractografía

Cervical Subependymoma Presenting as an Extramedullary Tumor

A rare case of cervical subependymoma in a 45-year-old man is described. The tumor appeared as an extramedullary mass lesion, but a discrete, less well-demarcated portion was observed in the anterolateral part of the cord at the C3-C7 level. Previous reports of spinal subepnedymomas are reviewed, and nosological possibilities of extramedullary presentation are discussed.

Subependymomas in children: a report of five cases including two with osseous metaplasia.

Subependymomas are highly differentiated slow growing gliomas. They are one of the few gliomas which are biologically benign. They are extremely rare in children. However, after going through the histopathology records of our department of fourteen years (1983-1997) we found that five (20%) cases of subependymomas have been diagnosed in children out of a total of twenty-six subependymomas. Two of our cases showed the presence of osseous metaplasia, a hitherto undescribed finding.

Various Tumors in the 4th Ventricle in Adults: MRI Findings

Primary fourth ventricular neoplasms are common in children but rare in adults, and the disease categories encountered differ according to the patient's age. This study reviewed the records of patients aged 16 years or over, who underwent magnetic resonance (MR) imaging and were found to have fourth ventricular lesions. Most patients then underwent surgical resection, leading to specific pathologic diagnosis. The various fourth ventricular tumors encountered were ependymoma (n=8), subependymoma (n=1), choroid plexus papilloma (n=3), astrocytoma (n=3), medulloblastoma (n=1), lymphoma (n=2), epidermoid cyst (n=2), meningioma (n=1), melanoma (n=1), cavernous hemangioma (n=1) and metastasis (n=1). We describe the various tumors located mainly in the fourth ventricle and review their clinical presentation and the radiological findings, the majority of which were nonspecific. In some cases, however, specific signal intensity or the growth pattern of the tumors was useful for differential diagnosis.

Immunohistochemical Study of Central Neurocytoma, Subependymoma and Subependymal Giant cell Astrocytoma Located around the Foramen of Monro

OBJECT: To gather information concerning ontogeny, the authors present the results of immunohistochemical stainings of neuronal and glial markers and the reverse transcriptase-prolongation chain reaction (RT-PCR) of nestin for three intraventricular tumors located around the foramen of Monro. METHODS: Seven cases of central neurocytomas(CN), three subependymomas(SE) and eight subependymal giant cell astrocytomas(SEGA), were included in this study. Antihuman monoclonal antibodies of synaptophysin(SNP)(DAKO, 1:20), chromogranin A(ChrA)(DAKO, 1:100), neuron specific enolase (NSE)(DAKO, 1:500) and nerve cell adhesion molecule(NCAM)(Zymed, 1:500) were utilized for neuronal markers and glial fibrillary acidic protein(GFAP)(DAKO, 1:300) functioned as a glial marker in immunohistochemical(IHC) stainings. Reverse transcriptase polymerase chain reaction(RT-PCR) for nestin was performed in all cases. RESULTS: For chromogranin A, positive reaction was found in three of the seven CN cases but none of the SE and SEGA cases. For IHC staining of synaptophysin, positive reaction was revealed in all CN cases but in none of the SE and SEGA cases. For NCAM, positive reaction was demonstrated in five of the eight SEGA cases and in all SE and CN cases. For NSE, positive reaction was exhibited in seven of the eight SEGA cases and in all SE and CN cases. Positive reactions for NSE and NCAM in the SEGA cases were manifested mainly in the cytoplasms of giant cells and their background. For IHC staining of GFAP, positive reaction was demonstrated in one of the seven CN cases, in three of the eight SEGA cases, and in all SE cases. RT-PCR product of nestin was expressed in two of the seven CN cases, in two of the three SE cases, and in one SEGA case. CONCLUSION: Many cells of CN, SE and SEGA, had expressed positive reactions for both neuronal and glial markers in IHC study and nestin in RT-PCR. It is suggested that origin cells of these tumors might express both neuronal and glial differentiation.

Subependimoma intraventricular / Intaventricular subependimoma

Se presenta el caso de una paciente de 52 años de edad quien desarrolló un tumor neuroepitelial clasificado como subependimoma del septum pellucidum con una expresión clínica exclusiva de síntomas psiquiátricos. Se intervino quirúrgicamente y se cuido la resección del borde inferior hacia el hipotálamo y subtálamo asistido con neuroendoscopía, la evolución de la paciente hasta hoy ha sido satisfactoria. La presentación de este tumor en la paciente permite la revisión en la historia y características de esta lesión descritas hasta este momento.

A Case of Normal Volume Hydrocephalus

The normal volume hydrocephalus is a serious, late complication of cerebrospinal fluid shunting procedures. It is characterized by persistent symptoms of headaches, vomiting and/or lethargy in shunted patients and is associated with normal-sized or slightly decreased ventricles. The subependymal gliosis that has been described in experimental and human hydrocephalus was offered as an explanation for the increased elastance in this condition. This 21-yearold male was shunted at 13 years of age for hydrocephalus secondary to pineal gland tumor. He remained asymptomatic for approximately 7 years before admission, when headaches and vomiting developed. A brain CT showed nearly normal-sized ventricles and spinal tapping documented elevated intracranial pressure (300mg CSF). A metrizamide shuntgram suggested obstruction of distal abdominal shunt catheter. After a revision of the distal shunt catheter, the patient became asymptomatic. On follow-up brain CT, no significant decrease in ventricle size was noted.

Sudden Respiratory Arrest after Surgery for Cerebellar Cystic Astrocytoma

The authors experienced a case of cystic cerebellar astrocytoma which showed sudden respiratory arrest after an uneventful operation. Preoperative cerebrospinal fluid diversion was not performed despite moderate hydrocephalus because we thought that complete removal of tumor enables the cerebrospinal fluid pathway to be reconstitute. After full awakening from anesthesia postoperatively, the patient's mentality deteriorated again rapidly with sudden respiratory arrest. Brain CT scan taken immediately after revealed no specific finding except moderate hydrocephalus which was the same degree as the preoperative one. This hydrocephalus was alleviated and the patient recovered slowly. We postulate several pathogenic mechanisms for this unusual event. First, chronic compression of fourth ventricle resulted in marked subependymal gliosis and obliteration of outlets of fourth ventricle. Therefore, postoperative reaccumlation of cerebrospinal fluid in ventricles caused serious pressure effect on the lower brain stem with resultant sudden respiratory arrest. Second, sudden decompression of brain stem might induce marked hemodynamic change in the brain stem. Third, there was some traction injury to brain stem by gravity in the sitting position. We suggest that preoperative cerebrospinal fluid diversion and its adeqaute postoperative maintenance is important in posterior fossa tumor surgery in cases with obliteration of perimesencephalic cistern and fourth ventricle, and with brain stem compression or angulation in preoperative magnetic resonance images.