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.
Glioma, Subependymal* ; Humans ; Middle Aged ; 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.
Adult ; Back Pain ; Female ; Glioma, Subependymal* ; Humans ; Leg ; Prognosis ; Radiotherapy ; Recurrence ; Spinal Cord* ; Spine

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.
Brain ; Catheters ; Cerebrospinal Fluid Shunts ; Follow-Up Studies ; Glioma, Subependymal ; Headache ; Humans ; Hydrocephalus* ; Intracranial Hypertension ; Lethargy ; Male ; Metrizamide ; Pinealoma ; Spinal Puncture ; Vomiting

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.
Adult ; Calcinosis/diagnosis ; Cerebellar Neoplasms/*diagnosis/surgery ; Fourth Ventricle ; Glioma, Subependymal/*diagnosis/surgery ; Humans ; Magnetic Resonance Imaging ; Male ; Rare Diseases/*diagnosis/surgery ; Tomography, X-Ray Computed

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.
Anesthesia ; Astrocytoma* ; Brain ; Brain Stem ; Cerebrospinal Fluid ; Decompression ; Fourth Ventricle ; Glioma, Subependymal ; Gravitation ; Hemodynamics ; Humans ; Hydrocephalus ; Infratentorial Neoplasms ; Tomography, X-Ray Computed ; Traction

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.
Antibodies, Monoclonal ; Astrocytoma* ; Cerebral Ventricles* ; Chromogranin A ; Cytoplasm ; Giant Cells ; Glioma, Subependymal* ; Immunohistochemistry ; Nestin ; Neural Cell Adhesion Molecules ; Neurocytoma* ; Neurons ; Phosphopyruvate Hydratase ; RNA-Directed DNA Polymerase ; Synaptophysin

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.
Astrocytoma ; Diagnosis ; Ependymoma ; Follow-Up Studies ; Glioma, Subependymal ; Humans ; Medical Records ; Radiotherapy, Adjuvant ; Recurrence ; Retrospective Studies ; Spinal Cord Neoplasms ; Spinal Cord ; Spine

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.
Adult* ; Astrocytoma ; Brain Neoplasms ; Child ; Diagnosis ; Diagnosis, Differential ; Ependymoma ; Epidermal Cyst ; Fourth Ventricle ; Glioma, Subependymal ; Hemangioma, Cavernous ; Humans ; Lymphoma ; Magnetic Resonance Imaging* ; Medulloblastoma ; Melanoma ; Meningioma ; Neoplasm Metastasis ; Papilloma, Choroid Plexus

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.
Adolescent ; Adult ; Brain ; diagnostic imaging ; pathology ; Child ; Child, Preschool ; Female ; Glioma, Subependymal ; diagnosis ; diagnostic imaging ; pathology ; Humans ; Lung ; diagnostic imaging ; pathology ; Magnetic Resonance Imaging ; methods ; Male ; Middle Aged ; Nervous System Diseases ; diagnosis ; diagnostic imaging ; pathology ; Tuberous Sclerosis ; classification ; diagnosis ; diagnostic imaging ; pathology