Intramedullary Subependymoma Of Lower Thoracic Cord.

Spinal subependymoma (SSE) is a rare intramedullary, benign tumour. Surgical excision isthe preferred approach. However, the interwoven pattern of neural tissue within the tumour dictates the extent of resection. Where gross total resection is linked with possible neurological deficits, subtotal resection or close observation may support better functional outcomes. The evidence for the management of SSE is based mostly on case reports. Herein, we review the existing literature regarding treatment options and clinical outcomes of spinal subependymoma.

Imaging characteristics of 4th ventricle subependymoma.

PURPOSE: Subependymomas located within the 4th ventricle are rare, and the literature describing imaging characteristics is sparse. Here, we describe the clinical and radiological characteristics of 29 patients with 4th ventricle subependymoma. METHODS: This is a retrospective multi-center study performed after Institutional Review Board (IRB) approval. Patients diagnosed with suspected 4th ventricle subependymoma were identified. A review of clinical, radiology, and pathology reports along with magnetic resonance imaging (MRI) images was performed. RESULTS: Twenty-nine patients, including 6 females, were identified. Eighteen patients underwent surgery with histopathological confirmation of subependymoma. The median age at diagnosis was 52 years. Median tumor volume for the operative cohort was 9.87 cm3, while for the non-operative cohort, it was 0.96 cm3. Thirteen patients in the operative group exhibited symptoms at diagnosis. For the total cohort, the majority of subependymomas (n = 22) were isointense on T1, hyperintense (n = 22) on T2, and enhanced (n = 24). All tumors were located just below the body of the 4th ventricle, terminating near the level of the obex. Fourteen cases demonstrated extension of tumor into foramen of Magendie or Luschka. CONCLUSION: To the best of our knowledge, this is the largest collection of 4th ventricular subependymomas with imaging findings reported to date. All patients in this cohort had tumors originating between the bottom of the body of the 4th ventricle and the obex. This uniform and specific site of origin aids with imaging diagnosis and may infer possible theories of origin.

A rare case of intraparenchymal subependymoma in a child.

Subependymoma is a slow-growing, exophytic, intraventricular glial neoplasm that commonly arises in the ventricular system. However, a report found that the frequency of intracerebral subependymoma was 0.4% in 1000 routine autopsies. To the best of our knowledge, only seven cases of intracerebral subependymoma have been reported. We report a rare case of intracerebral subependymoma in a child. An 11-year-old girl with generalized tonic-clonic seizures visited the emergency room and had an intraparenchymal tumor on the left frontal lobe on magnetic resonance imaging (MRI). Craniotomy with gross total removal was performed without any perioperative morbidities. The tumor was finally histopathologically diagnosed as a subependymoma.

Spinal cord subependymoma mimicking syringomyelia in a child: a case report.

Spinal cord subependymomas (SCSEs) in children are extremely rare, and no reports distinguishing SCSEs from syringomyelia have been published. We report a case of a 10-year-old boy who presented with torticollis, scoliosis, as well as pain that had begun in the posterior portion of the neck and progressed to the right shoulder and upper arm. Magnetic resonance imaging showed an intramedullary cyst-like lesion with the same signal intensity as that of cerebrospinal fluid. Idiopathic syringomyelia with scoliosis was first suspected, and a syrinx-subarachnoid space shunt was performed. After surgery, the lesion was slightly smaller; however, 2 years after surgery, it had re-grown, causing excruciating pain but no other symptoms. A second surgery was performed, and gross total resection was achieved. Pathological evaluation revealed SCSE. SCSE needs to be considered as a differential diagnosis for spinal centric cyst-like lesions in children.

Spinal subependymoma surgery: do no harm. Little may be more!

OBJECTIVE:  Outline the reported diagnostic and operative findings, and evaluate the surgical treatment outcome to clarify the best available recommendations. METHODS:  Ovid Medline, Embase and PubMed central databases were searched from inception until January 2019 using the terms (subependymoma and (spinal or cervical or thoracic)). The articles were reviewed for reported spinal subependymoma cases perioperative management and treatment outcomes. RESULTS:  A total of 49 papers provided data on 105 cases. 47 cases were reported in the last 5 years. The reported cases were two medullary-cervical, 35 cervical, 32 cervicothoracic, 21 thoracic, 12 thoracolumbar and three lumbar. Spinal subependymomas typically arise from within the central spinal canal, giving the appearance of an intramedullary mass, usually eccentric to one side. Symptoms at presentation ranged between 1 month to 17 years, (mean 3.5 years, median 2 years) and were over 3 years in 36, and over 8 years in 12 cases. Sensory symptoms are the most frequent 75(80%), followed by weakness in 60(64%), pain in 45(48%) and sphincter disturbance in 24(25%). Postoperative neurological function was reported in 78 cases, and worsening was reported in 40 cases (51%), of which, 29 (72%) had complete resection, 6 (15%) had subtotal resection and 5 (12%) had partial resection. Neurological status remained the same in 24 (30%) and improved in 14 (18%). CONCLUSION:  The reviewed cases report a rate of 65% total resection of which 57% had worsened function after surgery. There were no reports of malignant transformation; therefore, long-term survival is expected, and surgical caution should be exercised where there is minimal symptom progression.

Apoplexy of a collision tumour composed of subependymoma and cavernous-like malformation in the lateral ventricle: a case report.

Subependymomas are rare benign tumours arising from subependymal glial precursors that usually remain asymptomatic or may present due to obstruction of cerebrospinal fluid pathways. We describe the first report of intraventricular haemorrhage from subependymoma and cavernous-like malformation collision tumour in a 74-year-old male presented with an impaired level of consciousness.

Surgical management and long-term outcome of intracranial subependymoma.

BACKGROUND: Intracranial subependymomas account for 0.2-0.7% of central nervous system tumours and are classified as World Health Organization (WHO) grade 1 tumours. They are typically located within the ventricular system and are detected incidentally or with symptoms of hydrocephalus. Due to paucity of studies exploring this tumour type, the objective was to determine the medium- to long-term outcome of intracranial subependymoma treated by surgical resection. METHODS: Retrospective case note review of adults with intracranial WHO grade 1 subependymoma diagnosed between 1990 and 2015 at the Walton Centre NHS Foundation Trust was undertaken. Tumour location, extent of resection (defined as gross total resection (GTR), sub-total resection (STR) or biopsy) and the WHO performance status at presentation and through follow-up were recorded. RESULTS: Thirteen patients (7 males; 6 females) with a mean age of 47.6 years (range 33-58 years) and a median follow-up of 46 months (range 25-220 months) were studied. Eight patients had symptomatic tumours (headache, visual disturbance); five had incidental finding. Tumours were most commonly located in the fourth ventricle (n = 8). The performance status scores at diagnosis were 0 (n = 8) and 1 (n = 5). The early post-operative performance status scores at 6 months were 0 (n = 5) and 1 (n = 8) and at last follow-up were 0 (n = 11) and 1 (n = 2). There was no evidence of tumour re-growth following GTR or STR. The commonest complication was hydrocephalus (n = 3). CONCLUSION: Subependymoma are indolent tumours. No patients exhibited a worsening of performance status at medium- to long-term follow-up and there were no tumour recurrence suggesting a shorter follow-up time may be sufficient. Surgical resection is indicated for symptomatic tumours or those without a clear imaging diagnosis. Incidental intraventricular subependymoma can be managed conservatively through MRI surveillance.

[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.

Surgical outcomes in spinal cord subependymomas: an institutional experience.

Spinal cord subependymomas are very rare. Most studies on spinal cord subependymomas have been case reports with literature reviews. This study presented a surgical series of 13 patients with histologically proven spinal cord subependymomas. Their clinical data, radiological findings, operative records, and follow-up outcomes were reviewed. There were 5 male and 8 female patients with a mean age of 39.5 years. The mean follow-up period was 67.8 months. Four tumors were located in the cervical spine, 5 in the cervicothoracic spine, and 4 in the thoracic spine. Gross total resection (GTR) of the tumor with a well-demarcated dissection plane was achieved in 9 cases, and subtotal resection was achieved in 4 cases. The symptoms present before the surgery were improved in 11 cases at last follow-up and the current status of 2 patients had no change compared to the preoperative presentation at last follow-up. The postoperative follow-up magnetic resonance imaging showed no recurrence in the 9 GTR cases during the mean follow-up period of 70.3 months. No recurrence/regrowth of the residual tumors was observed in the 4 STR cases during the mean follow-up period of 62.0 months. Spinal cord subependymomas are amenable to surgical resection. It is possible to achieve GTR of intramedullary subependymomas that have a well-demarcated dissection plane. When GTR cannot be achieved, STR of the lesion for decompression is advised, and follow-up imaging is needed. A good clinical outcome after GTR or STR can be expected.

Surgical resection of subependymoma of the cervical spinal cord.

Subependymomas can rarely occur in the spinal cord, and account for about 2% of symptomatic spinal cord tumors. It most often occurs in the cervical spinal cord, followed by cervicothoracic junction, thoracic cord and conus medullaris. It often has an eccentric location in the spinal cord and lacks gadolinium enhancement on magnetic resonance imaging. We present a rare case of symptomatic subependymoma of the cervical spinal cord, which underwent successful gross total resection. Surgical pearls and nuances are discussed to help surgeons to avoid potential complications. The video can be found here: http://youtu.be/Rsm9KxZX7Yo.

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.

Thoracolumbar intramedullary subependymoma with multiple cystic formation: a case report and review.

BACKGROUND: An intramedullary subependymoma is rare, particularly in the thoracolumbar region. Moreover, a radiographical obvious cystic formation of subependymoma of spinal cord rarely occurs in comparison to ependymoma. CASE REPORT: A 57-year-old woman presented with paraparesis. MRI revealed a multinodular and multicystic lesion in the spinal cord that was difficult to diagnose correctly. Intraoperative observation via midline myelotomy revealed a grayish, gelatinous solid mass with an eccentric localization. In addition, DREZtomy on the caudal side of the tumor revealed cystic formation. The cyst was punctured and xanthochromic fluid was collected. Attenuation of motor evoked potential (MEP) resulted in the partial removal of tumor. A pathological examination revealed the mass to be a subependymoma. The patient experienced transient worsening of symptoms, but improved gradually. No adjuvant radiosurgery was administered. Follow-up estimation 30 months after surgery revealed no evidences of regrowth. CONCLUSIONS: This report presents this rare case, a review of the literature associated with thoracolumbar subependymomas, and a discussion of the clinical and radiographical characteristics.

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.

The Ribbon Sign as a Radiological Indicator of Intramedullary Spinal Cord Subependymomas.

OBJECTIVE: Intramedullary spinal cord (IMSC) subependymomas are rare World Health Organization grade 1 ependymal tumors. The potential presence of functional neural tissue within the tumor and poorly demarcated planes presents a risk to resection. Anticipating a subependymoma on preoperative imaging can inform surgical decision-making and improve patient counseling. Here, we present our experience recognizing IMSC subependymomas on preoperative magnetic resonance imaging (MRI) based on a distinctive characteristic termed the "ribbon sign." METHODS: We retrospectively reviewed preoperative MRIs of patients presenting with IMSC tumors at a large tertiary academic institution between April 2005 and January 2022. The diagnosis was confirmed histologically. The "ribbon sign" was defined as a ribbon-like structure of T2 isointense spinal cord tissue interwoven between regions of T2 hyperintense tumor. The ribbon sign was confirmed by an expert neuroradiologist. RESULTS: MRIs from 151 patients were reviewed, including 10 patients with IMSC subependymomas. The ribbon sign was demonstrated on 9 (90%) patients with histologically proven subependymomas. Other tumor types did not display the ribbon sign. CONCLUSION: The ribbon sign is a potentially distinctive imaging feature of IMSC subependymomas and indicates the presence of spinal cord tissue between eccentrically located tumors. Recognition of the ribbon sign should prompt clinicians to consider a diagnosis of subependymoma, aiding the neurosurgeon in planning the surgical approach and adjusting the surgical outcome expectation. Consequently, the risks and benefits of gross-versus subtotal resection for palliative debulking should be carefully considered and discussed with patients.

Subependymomas of the fourth ventricle: To operate or not to operate?

BACKGROUND: There is a paucity of literature regarding the clinical characteristics and management of subependymomas of the fourth ventricle due to their rarity. Here, we describe the operative and non-operative management and outcomes of patients with such tumors. METHODS: This retrospective single-institution case series was gathered after Institutional Review Board (IRB) approval. Patients diagnosed with a subependymoma of the fourth ventricle between 1993 and 2021 were identified. Clinical, radiology and pathology reports along with magnetic resonance imaging (MRI) images were reviewed. RESULTS: Patients identified (n = 20), showed a male predominance (n = 14). They underwent surgery (n = 9) with resection and histopathological confirmation of subependymoma or were followed with imaging surveillance (n = 11). The median age at diagnosis was 51.5 years. Median tumor volume for the operative cohort was 8.64 cm3 and median length of follow-up was 65.8 months. Median tumor volume for the non-operative cohort was 0.96 cm3 and median length of follow-up was 78 months. No tumor recurrence post-resection was noted in the operative group, and no tumor growth from baseline was noted in the non-operative group. Most patients (89 %) in the operative group had symptoms at diagnosis, all of which improved post-resection. No patients were symptomatic in the non-operative group. CONCLUSIONS: Surgical resection is safe and is associated with alleviation of presenting symptoms in patients with large tumors. Observation and routine surveillance are warranted for smaller, asymptomatic tumors.

Third Ventricular Subependymomas: Clinical Features and Outcomes Over Two Decades.

BACKGROUND: Subependymomas are uncommon, benign slow-growing neoplasms of the central nervous system preferentially arising within the fourth and lateral ventricles. Third ventricle involvement has been described rarely. The aim of this study is to provide the first systematic review of third ventricular subependymomas (TVSE) by analyzing all reported cases over 2 decades and describing a case example. METHODS: MEDLINE and Embase databases were searched for the 20 years ending January 1, 2022, using relevant MeSH and non-MeSH terms, including "subependymoma" and "third ventricle." Methodology followed PRISMA guidelines. RESULTS: Of 804 identified studies, 131 met inclusion eligibility. The literature yielded 17 patients with TVSE plus our example (18 total). Of these patients, 83% (15/18) presented in adulthood (average age, 42 ± 19 years), of whom 73% were women. The pediatric cohort age was 5 ± 1 years, 67% (4/6) of whom were girls. The most common presenting symptom in both cohorts was headache (80%), followed by memory disturbances and vomitus. In adults, symptomatic tumors were approached by open craniotomy in all but 1 case, most using a transcallosal approach. Gross total resection was obtained in 73%. A ventriculoperitoneal shunt was inserted in 2/15 adult and 4/6 pediatric patients. Overall, both cohorts showed symptomatic improvement without disease recurrence. One patient died perioperatively. CONCLUSIONS: Subependymomas should be considered in the differential diagnosis of third ventricular tumors. The clinical presentation of TVSE mainly parallels hydrocephalus symptoms and, hence, awareness is of vital importance for timely treatment. The surgical goal should be gross total resection, which can be curative and offers greatest clinical improvement across the population.

Cerebellopontine angle subependymoma without fourth ventricle extension: an uncommon tumor in a rare location.

Subependymomas are benign tumors that occur predominantly in the ventricular system. We describe a case of a 57-year-old man with a large cerebellopontine angle (CPA) tumor which expanded into the jugular foramen. Complete surgical excision of the tumor was achieved through a retrosigmoid approach and the histopathological diagnosis was subependymoma. Subependymomas located exclusively in the CPA without extension into the fourth ventricle are extremely rare. The mainly pathological features and the difficulty in correctly diagnosing these cases preoperatively, even with MRI, are discussed.

Lateral ventricular subependymomas: An analysis of the clinical features of 27 adult cases at a single institute.

OBJECTIVE: To evaluate the unique clinical characteristics and management of lateral ventricular subependymomas (LVSs). Patients and Methods : The case records of 27 adult consecutive patients with LVS admitted between March 1996 and May 2011 were reviewed. The relevant clinical data (including patient age and sex, neuroimaging studies, surgical records and follow up) were collected through a chart review. Patient neurological status was recorded using the Karnofsky Performance Scale (KPS). RESULTS: The gender distribution was 14:13 and the age from 33 to 66 years (median 45 years) at the time of operation. Headache and dizziness were the most common initial symptoms (17/27). Most of these tumours were located at the foramen of Monro (12/27). Magnetic resonance imaging (MRI) (21/27) showed well circumscribed tumours with cystic changes (21/27). The lesions were hypointense on T1-weighted images (19/21), hyperintense on T2-weighted images (21/21), and contrast enhancement was no or minimal (19/21). Gross total resection was performed in 23 patients. Five patients required a ventriculo-peritoneal shunt because of postoperative hydrocephalus. The follow-up period ranged from 6 to 188 months (mean 55.5 months). No recurrence was observed during the follow up. CONCLUSION: In this study LVSs had equal gender distribution. Tumours around the foramen of Monro were the candidates for aggressive treatment; surgery was the best curative treatment; postoperative hydrocephalus should be attended to.

Tumor characteristics and surgical outcomes of intracranial subependymomas: a systematic review and meta-analysis.

OBJECTIVE: The tumor characteristics and surgical outcomes of intracranial subependymomas are poorly defined. In this study the authors aimed to provide a comprehensive review of all clinical, pathological, radiological, and surgical aspects of this important neoplasm to inform future management strategies. METHODS: A systematic review and meta-analysis of MEDLINE, EMBASE, Cochrane, and Google Scholar databases adherent to PRISMA guidelines was conducted. RESULTS: Of the 1145 articles initially retrieved, 24 studies encompassing 890 cases were included. The authors identified 3 retrospective cohort studies and 21 case series, but no controlled trials. Mean age at presentation was 46.7 ± 18.1 years with a male predominance (70.2%). Common sites of tumor origin were the lateral ventricle (44.5%) and fourth ventricle (43.1%). Cumulative postoperative mortality and morbidity rates were 3.4% and 24.3% respectively. Meta-analysis revealed that male sex (HR 3.15, 95% CI 1.39-7.14, p = 0.006) was associated with poorer 5-year overall mortality rates. All-cause mortality rates were similar when performing subgroup meta-analyses for age (HR 0.50, 95% CI 0.03-7.36, p = 0.61), smaller subependymoma size (HR 1.51, 95% CI 0.78-2.92, p = 0.22), gross-total resection (HR 0.65, 95% CI 0.35-1.23, p = 0.18), and receipt of postoperative radiation therapy (HR 0.88, 95% CI 0.27-2.88, p = 0.84). Postoperative Karnofsky Performance Index scores improved by a mean difference of 1.62 ± 12.14 points (p = 0.42). The pooled overall 5-year survival rate was 89.2%, while the cumulative recurrence rate was 1.3% over a median follow-up ranging from 15.3 to 120.0 months. The pure subependymoma histopathological subtype was most prevalent (85.6%), followed by the mixed subependymoma-ependymoma tumor variant (13.7%). CONCLUSIONS: Surgical extirpation without postoperative radiotherapy results in excellent postoperative survival and functional outcomes in the treatment of intracranial subependymomas. Aggressive tumor behavior should prompt histological reevaluation for a mixed subependymoma-ependymoma subtype. Further high-quality controlled trials are still required to investigate this rare tumor.