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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

Subependymoma of the Conus Medullaris with Cystic Formation: Case Report and a Literature Review.

BACKGROUND: Subependymoma in the spinal cord is very rare and usually occurs in the cervical cord. We report an exceptional case of subependymoma that occurred at the conus medullaris with cystic formation. This article reviews the literature on subependymoma in the conus medullaris; discusses its clinical manifestations, imaging findings, and differential diagnoses; and offers an opinion about the cystic formation of the subependymoma. CASE DESCRIPTION: A 69-year-old woman experienced progressive limb weakness with a somatosensory abnormality for 3 months. Preoperative magnetic resonance imaging showed a cystic intramedullary lesion at the conus medullaris with a well-defined margin. A preliminary diagnosis of epidermoid cyst was made based on the imaging findings. During the operation, cystic formation of the tumor was found, and the tumor was completely removed. Pathology showed an uneven proliferation of glial cells, consistent with subependymal morphology, and the tumor was confirmed as subependymoma. CONCLUSIONS: We present an extremely rare case of cystic formation in subependymoma at the conus medullaris. Subependymoma should be included in the differential diagnosis of intramedullary cystic lesions. The breakdown of the blood-brain barrier and excessive extravasation may be potential mechanisms of cystic formation.

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.

Microsurgical Resection of the IV Ventricle Subependymoma: 2-Dimensional Operative Video.

In this video, we demonstrate microsurgical resection of IV ventricle subependymoma. To the best of our knowledge, this is the first video case report of a microsurgical resection of subependymoma of the IV ventricle in the peer-reviewed English literature. Subependymomas are benign central nervous system tumors, typically arising in ventricular spaces, mostly in the IV and lateral ventricles.1-3 They are isointense on T1 and hyperintense on T2-weighted magnetic resonance imaging (MRI) with minimal or no enhancement.4 Microsurgery remains the mainstay treatment. Complete tumor resection is possible and curative with excellent prognosis.1,5-7 Although the clinical course appears benign, the inability to diagnose them radiographically with certainty and the possibility of an alternative malignant lesion support a low threshold for early and safe resection.8 A 39-yr-old man presented with severe headache and balance problems. Pre- and postcontrast neuroaxis MRI revealed a centrally located IV ventricle lesion without hydrocephalus. The aim of the surgery was complete tumor resection. Surgery was performed in the prone position by the senior author (KIA) with intraoperative neurophysiology monitoring. A small suboccipital craniotomy and C1 posterior arch removal was done. After opening the dura and arachnoid membrane, the tumor was identified and meticulously dissected from the adjacent posterior inferior cerebellar artery and the floor of the fourth ventricle and from brain stem white matter at the tumor-neural tissue interface to avoid brainstem interference. Histological analysis revealed subependymoma (World Health Organization Grade I). Postoperative pre- and postcontrast MRI revealed complete resection. Headache and balance problems completely resolved; the patient was neurologically intact. The patient provided written consent and permission to publish his image.

Unusual Exophytic Appearance of Spinal Cord Subependymoma.

BACKGROUND: Subependymomas are rare in the spinal cord. They are typically expansile, intramedullary spinal cord masses, eccentrically located with minimal gadolinium enhancement. CASE DESCRIPTION: We present a case of subependymoma originating from the cervical cord with an unusual exophytic appearance. Hallmarks of subependymoma and treatment are reviewed. CONCLUSIONS: This is the first case, to our knowledge, where imaging revealed a mass appearing to be completely extramedullary with a primary exophytic component. Therefore, subependymomas should remain on the differential for masses in the spinal cord that appear extramedullary and exophytic.

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.

Flexible Fiber CO2 Laser in Microsurgical Treatment of Intraventricular Tumors: Usefulness and Limitations.

OBJECTIVE: To assess usefulness and limitations of flexible fiber carbon dioxide (CO2) laser in the microsurgical treatment of intraventricular tumors. METHODS: We reviewed a series of 9 patients treated with microsurgical resection of intraventricular tumors using a flexible fiber CO2 laser. The lesions involved the third ventricle (8) and the frontal horn of the right lateral ventricle (1). Histology revealed 6 craniopharyngiomas, 1 pituitary macroadenoma, 1 subependymoma, and 1 neurocytoma. In all cases, an interhemispheric transcallosal approach was performed. The laser was used during callosotomy, fornix column sectioning, tumor debulking, and to facilitate tumor dissection. We used a 5-tiered score system to assess laser's efficacy in each surgical step (approach, dissection, debulking): grade 1: laser was not at all helpful, grade 5: laser was extremely helpful. Limits of the instrument also are discussed. RESULTS: Gross total resection was achieved in 6 cases and subtotal resection in the remaining 3. Three patients had pulmonary complications treated without clinical sequelae. No laser-related complication was described. Mean utility score observed was 4.2 (range 3-5) during approach, 2.8 (range 2-4) during tumor dissection; and 3.3 (range 2-5) during tumor debulking. Main limits were low hemostatic effect and inefficiency versus calcified and highly vascularized tumors. CONCLUSIONS: The CO2 laser proved to be a useful and safe tool that could be used for intraventricular pathology; its design is suitable for narrow surgical corridors like interhemispheric fissure and foramen of Monro; its main utility is the ability to create precise and relatively bloodless cut (callosotomy, tumor debulking); low hemostatic effect is its main limit.