A Case of Cerebellar Intraparenchymal Subependymoma with Next-Generation Sequencing Analysis.

Subependymomas are rare, intraventricular glial tumors histologically characterized by clusters of small uniform cells distributed in an abundant fibrillary matrix. These tumors can arise in the parenchyma of the cerebrum, cerebellum, or spinal cord. Herein, we report an extremely rare case of cerebellar intraparenchymal subependymoma in a 62-year-old woman. The patient presented with dizziness for several years, and brain magnetic resonance imaging revealed a well-defined solid mass in the right cerebellum, upon which a stereotactic biopsy was performed. Histologically, the tumor showed a distinctive multinodular pattern with unevenly distributed glial cells and an abundant fibrillary matrix. Next-generation sequencing analysis showed balanced genomes without genetic alterations, including single-nucleotide variants, small insertions, deletions, or copy number alterations. Follow-up magnetic resonance imaging revealed that the size of the mass has not changed; the patient has not received any surgical treatments since the pathologic diagnosis and is living healthily.

A case of chronic dysaesthesia in the torso and upper limbs: lessons from a cervical spinal cord subependymoma.

INTRODUCTION: Subependymomas are slow growing WHO grade 1 tumours, typically attached to the ventricular wall of the fourth or lateral ventricles. Spinal subependymomas are rarer still and experience of their biological characteristics remains limited. CASE PRESENTATION: A 29-year-old lady presented with chronic attacks of itchy dysaesthesia involving the left hand, neck and trunk, and associated with ipsilateral leg spasms. Recent symptomatic change involved occasional limping and left sided facial numbness but no pain. MRI showed an intradural mass surrounding most of the cervical spinal cord, which appeared scalloped extrinsically, rather than diffusely expanded, by a seemingly extramedullary lesion. At operation, the cord appeared expanded, with no clear margin or distinction between tumour and cord tissue; and the tumour was found to be intramedullary with an exophytic component, rather than extramedullary. Moderate reduction of the left abductor pollicis brevis evoked potential led to a pause in surgery. There was transient hand weakness postoperatively with full recovery, and no radiological change in the tumour morphology for a further 6 years. DISCUSSION: An intramedullary tumour such as a spinal cord subependymoma can be mistaken radiologically for an extramedullary tumour, such as an epidermoid. If a subependymoma is suspected, given its indolent course and long-term survival, caution in the extent of surgical resection is advisable in order to avoid surgical morbidity.

Is This Truly A "Leave-Me-Alone" Lesion? An Unusual Case of Multiple Ring-shaped Lateral Ventricular Nodules.

BACKGROUND: Ring-shaped lateral ventricular nodules (RSLVNs) are commonly considered as benign asymptomatic lesions, which are sporadically detected as incidental findings on routine brain magnetic resonance imaging scans. Despite their not irrelevant frequency, the exact biological nature of these lesions remains largely unknown due to the lack of histopathologic studies. Here we present the clinical, neuroradiologic, and histopathologic findings of an unusual case of symptomatic multiple RSLVNs. CASE DESCRIPTION: A 44-year-old otherwise healthy man presented with a recent history of headache and retching. Neuroradiologic imaging revealed the presence of multiple RSLVNs, the largest of which, located in the cella media of the right lateral ventricle, exerted a mild to moderate mass effect on adjacent brain parenchyma. This latter nodule was successfully removed, with complete resolution of the symptoms. Histopathology revealed glial differentiation, and the specimen was diagnosed as subependymoma. CONCLUSIONS: This report provides novel evidence characterizing RSLVNs as possible variants of subependymoma with a peculiar imaging appearance, also suggesting that, like subependymomas, they may occasionally grow large enough to cause mass effect-related symptoms, thus requiring neurosurgical intervention.

Squash cytology findings of subependymomas: A report of three cases and differential diagnosis.

Subependymomas are slowly growing glial tumors, corresponding to WHO grade I. Few descriptions of the cytologic features of this neoplasm are available. This study describes the cytologic features of three subependymomas, as well as their differential diagnosis based on cytology. Three men, aged 52, 56, and 63 years, presented with headache. Magnetic resonance imaging revealed a nodular intraventricular mass in all three patients. Intraoperative squash cytology specimens from the three intraventricular tumors showed nodular clusters with microcystic changes. Nuclei were round to oval in shape, but showed no evidence of severe nuclear atypia or mitoses. Histological examination showed features of subependymoma. Squash cytology findings, including nodular clusters, mild cellular atypia, microcystic changes, and mucoid material, are useful in the rapid intraoperative diagnosis of subependymoma.

Multiple supratentorial subependymomas causing obstructive hydrocephalus.

INTRODUCTION: Subependymomas are benign intraventricular tumours that most often occur asymptomatically and are found incidentally on autopsy. Symptomatic examples requiring surgical intervention are exceedingly rare. CASE PRESENTATION: A 55-year-old man with no history of neurological symptoms presented with multiple episodes of loss of consciousness and increasing headaches. MRI revealed a lobulated intraventricular mass centred at the right Foramen of Monro. Obstructive hydrocephalus with localised midline shift and a second lesion were noted. Right frontal craniotomy with total removal via transcortical resection was performed. DISCUSSION: Symptomatic subependymomas generally present with signs of hydrocephalus due to obstruction of cerebrospinal fluid pathways. There is only one other reported case of multifocal subependymomas in a symptomatic patient. An example of multiple supratentorial subependymomas causing obstructive hydrocephalus has not been previously reported. CONCLUSIONS: Multiple subependymomas are rare. Judicious surgical management with full excision led to symptomatic improvement in our patient.

Tuberous sclerosis complex: Imaging characteristics in 11 cases and review of the literature.

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.

Findings from frozen sections of spinal subependymomas: Is it possible to differentiate this diagnosis from other common spinal tumors?

Subependymomas are slow-growing, benign neoplasms that are rarely found in the spinal cord. Because of the differences in the treatment plans, it might be very helpful for neurosurgeons to intraoperatively establish a diagnosis of spinal subependymoma, differentiated from other spinal intramedullary tumors. In this study, we analyzed frozen sections of spinal subependymomas to identify potential histological clues of spinal subependymomas to differentiate them from tumors that mimic spinal subependymoma. We reviewed the frozen sections and the corresponding permanent slides for 7 cases of spinal subependymoma. The spinal subependymomas showed several characteristic patterns, including, most importantly, an eccentric or both central and eccentric location in the axial plane. Histologically, they showed a (1) well-demarcated and multinodular mass with (2) low or moderate cellularity, (3) a microlobular pattern, and (4) small clusters of neoplastic cells. These features appear to be very specific to spinal subependymomas and could help differentiate them from ependymomas or astrocytomas. Although we might not be able to provide an exact diagnosis of all spinal subependymomas using these histological features, we hope that they help neuropathologists and neurosurgeons to adequately diagnose and treat spinal subependymomas.

[A Rare Case of Subependymoma of the Septum Pellucidum as Intratumoral Hemorrhage].

Subependymomas (SEs) are rare, benign, noninvasive, slow-growing tumors located anywhere along the ventricular walls. They arise most frequently in the fourth ventricle followed by the lateral ventricle, and less frequently in the septum pellucidum, third ventricle, and spinal cord. Most SEs are found incidentally at autopsy, but some may produce clinical symptoms. Tumor-related hemorrhage represents an extremely rare presentation sign. We describe a rare case of septum pellucidum SE as tumoral hemorrhage. The tumor was totally removed via an interhemispheric transcallosal approach. Histological examination found typical SE. Although the patient had transient memory impairment, he had a good postoperative course and was discharged on the twenty-first postoperative day.

Simultaneous subependymomas in monozygotic female twins: further evidence for a common genetic or developmental disorder background.

In this paper, a rare case of subependymoma of the fourth ventricle in identical female twins is reported. Magnetic resonance imaging and CT showed nearly identical locations of the tumors in the fourth ventricle and similar growth patterns of the tumors in both sisters. Likewise, postoperative histopathological analysis of both tumors revealed the typical histological appearance of subependymomas. Subependymoma is a rare, low-grade glioma of the CNS, slowly growing and usually asymptomatic. If symptomatic, a subependymoma can in some cases lead to sudden death caused by pressure on the brainstem or decompensated secondary hydrocephalus. This case demonstrates the importance of detecting tumors early and thereby preventing symptoms arising from increasing intracranial pressure, and optimizing therapy options.

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.

Symptomatic subependymomas of the ventricles. Review of twenty consecutive cases.

BACKGROUND AND PURPOSE: Intraventricular subependymomas are rare benign tumors, which are often misdiagnosed as ependymomas. To review the clinicopathological features of subependymomas. PATIENT SELECTION AND METHODS: Retrospective clinical analysis of intraventricular subependymomas and systematic review of histological slides operated on at our center between 1985 and 2005. RESULTS: Twenty subependymomas presented at the median age of 50 years (range 19-77). Two (10%) were found in the third, three (15%) in the forth, and 15 in the lateral ventricles. There was male preponderance (12 vs. 8). Ataxia (n=13) and papilledema (n=7) were the most common clinical presentations. Fifteen patients underwent gross total resection, and five had subtotal resection. None of the cases showed mitotic figures, vascular endothelial proliferation or necrosis. Cell proliferation marker MIB-1 activity (percentage of positive staining tumor cells) ranged from 0 to 1.4% (mean 0.3). Two cases were treated with preoperative radiation therapy (50 Gy) before the CT era, three other patients received postoperative radiation therapy for tumors originally diagnosed histologically as low grade ependymomas. Three patients (15%) died of surgical complication between one and three months postoperatively, and three patients died of unrelated causes in eight, 26 and 110 months. Fifteen patients were alive without evidence of tumor recurrence at a median follow-up time of 10 years. CONCLUSION: Subependymomas are low-grade lesions and patients do well without adjuvant radiotherapy. Small samples from more cellular areas may be confused with low grade ependymomas, and unnecessary radiotherapy may follow. Recurrences, rapid growth rates should warrant histological review, as hypocellular areas of ependymomas may also be a source of confusion.

Interesting case of subependymoma of the spinal cord.

BACKGROUND CONTEXT: Subependymomas are rare, slow-growing, and usually noninvasive/nonaggressive World Health Organization Grade I tumors that tend to occur in the ventricles. Their most common site of occurrence is the fourth ventricle followed by the lateral ventricles. Spinal cord subependymomas typically manifest as cervical and cervicothoracic intramedullary or, rarely, extramedullary mass lesions. They often present clinically with pain and neurologic symptoms, including motor, sensory, urinary, and sexual dysfunction. Histologically, there are hypocellular areas with occasional clusters of cells and frequent microcystic changes, calcifications, and hemorrhage. Radiologically, subependymomas generally manifest as eccentric well circumscribed nodular lesions with mild-to-moderate enhancement. PURPOSE: To highlight an interesting and rare presentation for subependymoma of the spinal cord. STUDY DESIGN: This is a case report of a single patient in whom a subependymoma was resected from the cervical spinal cord with return to normal functioning. METHODS: Clinical examination, magetic resonance imaging evaluation, surgical resection, and histological analysis were performed for diagnosis and treatment of this patient. RESULTS: The patient experiencing myelopathy symptoms underwent a surgical resection of cervical spinal cord subependymoma that resulted in return to normal function. CONCLUSIONS: Subependymoma should be included in the differential diagnosis of atypical presentations for myelopathy, as discrete surgical resection can result in good outcome.

Atypical hemorrhagic presentation of a fourth ventricle subependymoma: case report.

OBJECTIVE: To present a case of a fourth ventricle subependymoma (SE) with a spontaneous acute subarachnoid intra-cisternal bleeding. METHODS: A 33-year-old man was admitted with 5 days history of oppressive occipital headache and neck pain without additional neurological focus. Unenhanced computed tomography (CT) scan demonstrated an isointense mass located in the fourth ventricle with a spontaneously hyperdense acute extratumoral hemorrhage in the cisterna magna. Contrast-enhanced magnetic resonance imaging (MRI) revealed a well-delimitated non-enhanced tumor, hypointense on T1-weighted and hyperintense on T2-weighted images, involving the floor of the fourth ventricle and extending caudally into the cervical spinal canal via foramen magnum. RESULTS: Intraoperative, a large blood clot was removed and a macroscopically hypovascularlesion was completely excised from the right lateral recess and the floor of the fourth ventricle. Intra and postoperative immuno-histopathological examination revealed a SE. The patient has a normal postoperative course and was discharged in the fifth postoperative day. A 10-month postoperative MRI study confirmed a complete tumor resection. CONCLUSION: Symptomatic SEs should be surgically treated emphasizing the urgency in the presence of hemorrhage. The interest of this case is to demonstrate that infratentorial SEs although extremely rare, might present with acute subarachnoid bleeding.

[Subependymoma of the lateral ventricle. A case report]. / Subependimoma del ventriculo lateral. A proposito de un caso.

INTRODUCTION. Intracranial subependymomas are rare, slow-growing, noninvasive, benign tumors. They are most often located in the fourth ventricle. Most of these tumors are discovered incidentally during autopsy. Routine medical checkups using neuroimaging techniques have increased their diagnosis. Subependymomas may present with symptoms related to cerebrospinal fluid obstruction or mass effect. CASE REPORT. A 52-year-old man presented with severe headache and mental deterioration with memory disturbances and bradypsychia. Computed tomography and magnetic resonance imaging revealed a mass in the right lateral ventricle causing obstructive hydrocephalus. The tumour was totally removed through a right frontal transcortical approach. Histological examination showed a typical subependymoma. A complete neurological recovery was achieved after surgery. CONCLUSIONS. Subependymomas are rare low-grade glial neoplasm that commonly arise in the ventricular system. They have a low-proliferative potential but in these locations they can cause symptomatic hydrocephalus. Surgical removal of the mass and the restoration of the normal cerebrospinal fluid pathways constitute the optimal management strategy.

Subependymal spread of recurrent glioblastoma detected with the intraoperative use of 5-aminolevulinic acid: case report.

Recurrent glioblastoma (GBM) can occur locally or at distant sites within the brain. Though MRI is the standard imaging modality for primary and recurrent GBM, the full extent of diffuse lesions may not be appreciated on MRI alone. Glioblastomas with ependymal and/or subependymal spread are examples of diffuse infiltrative tumors that are incompletely seen on MRI. Some other adjuvant visualization technique such as intraoperative fluorescence-assisted 5-aminolevulinic acid (5-ALA) could be used to assist the surgeon in localizing the infiltrating tumor. The authors report on a 56-year-old man who presented 7 years after initial resection of an occipital lobe GBM with imaging consistent with distant discrete foci of tumor recurrence. Because these foci were distant from the original resection cavity, there was concern for diffuse, infiltrative tumor elsewhere throughout the brain versus a distant multicentric recurrence. Therefore, the patient was given 5-ALA prior to surgery to aid in tumor detection intraoperatively. Using fluorescent visualization of the resection cavity, it was confirmed that there was subependymal and ependymal spread of the recurrent tumor along the lateral ventricle connecting the recurrence to the previous tumor site. Magnetic resonance imaging may not completely detect the presence of diffuse tumor infiltrating the ependymal or subependymal spaces. Therefore, adjunct intraoperative use of fluorescence-assisted visualization with 5-ALA may be helpful in highlighting and detecting infiltrative tumor to accurately detect tumor burden and distinguish it from a separate multicentric recurrence.