Neurenteric Cyst Research Articles

Neurenteric cyst of right posterior mediastinum- A case report

Neurenteric cysts are rare congenital abnormalities, commonly occurring in the spinal region, composed of heterotopic endodermal tissue components. We present a case of six months child having cough and intermittent fever. Computed tomography scan showed presence of cystic structure in the mediastinal region. Microscopy showed cyst wall lined by gastric type of mucosa with mucinous secretion lying in the cystic space. Neurenteric cysts are infrequent findings in routine medical practice. There is scanty literature, in relation to its diagnosis, management, and long-term outcome.

Endoscope-Assisted Resection of Extra-Axial Premedullary Neurenteric Cyst via Far Lateral-Supracondylar Approach: 2-Dimensional Operative Video.

Neurenteric cysts are rare congenital lesions that may compress the ventral brainstem.1-9 In this operative video, we illustrate the surgical treatment of an intradural extra-axial neurenteric cyst extending from the lower pons to the craniocervical junction. The patient, an asymptomatic 52-yr-old female, underwent surveillance imaging of the premedullary lesion for 14 yr without progression. However, after developing progressive strain-induced headaches, imaging revealed a significant enlargement of the lesion with brainstem compression and partial obstruction of the foramen magnum. Therefore, surgical resection was pursued. The patient consented to the procedure. The patient underwent a lateral suboccipital craniotomy and C1 laminectomy through a far lateral approach. The lesion was immediately visualized upon opening the dura. After identifying the cranial nerves, we resected the tumor while taking care to preserve the neurovascular elements of the cerebellopontine angle and foramen magnum. During the resection, we unexpectedly encountered a firm nodule that was adherent to the right posterior inferior cerebellar artery. This was meticulously dissected and removed en bloc using intraoperative indocyanine green (ICG) angiography. The cavity was inspected with 0-degree and 30-degree endoscopes to ensure complete resection of the lesion. Gross total resection was confirmed on postoperative magnetic resonance imaging. The patient was neurologically intact with no cranial nerve abnormalities and discharged home on postoperative day 3. This case demonstrates that the far lateral-supracondylar approach affords safe access to the ventral pontomedullary and craniocervical junctions and that intraoperative adjuncts, including ICG angiography and endoscopic visualization, can facilitate complete lesion resection with excellent clinical outcomes.

Spinal intradural endodermal cyst

Purpose: Epithelial cysts of the central nervous system, according to the type of cell origin; neuroepithelial or endodermal type. Endodermal cysts are rare forms of benign tumors. In this presentation, we present a 35-year-old male patient with typical histopathologic features and due to the presence of this rare lesion. We aimed to present this rare case and emphasize the importance of cystic epithelial cell differences. Methods: Neurenteric cysts, cyst lining epithelium of cell types were analyzed using immunohistochemical diagnostic methods. Results: The importance of the cell type in the endodermal cyst is also emphasized and the frequency of recurrence in cysts that have goblet-columnar epithelial component. This epithelium is increasing the risk of recurrences. However, the other cause of recurrence is also incomplete excision. Therefore, the complete excision of ECs is important, not only for avoiding recurrence but also for differentiating cell types for ECs and predicting recurrence risk. Conclusions: The precise pathologic diagnosis of cell type determines the risk of recurrence. Neurosurgeons should exert their best efforts to achieve complete excision of the cysts.

Predictors for complete surgical resection of posterior fossa neurenteric cysts: A case report and meta-analysis.

Background: Incomplete resection of neurenteric cysts (NCs) has been associated with increased recurrence rates in patients compared to complete resection (CR) and information on intracranial NCs appearance on diagnostic imaging is scarce. We sought to identify factors associated with CR and provide the largest up-to-date review of NCs appearances on various diagnostic images.Methods: Data from Medline, EMBASE, and Web of Science were extracted. Univariate and multivariate logistic regression models were used to analyze factors associated with CR.Results: A total of 120 publications reporting 162 original cases on posterior fossa NCs met the inclusion criteria for analysis. Eighty-nine (55.6%) of the patients were female, the mean (SD) age of the patients’ during operation was 34.3 (16.9) years, and CR was achieved in 98 (60%) of patients. Univariate analysis identified male sex as a statistically significant predictor for complete reaction (OR 2.13, 95% Cl 1.10–4.11, P = 0.02). The retrosigmoid approach (OR 1.89, 95% Cl 0.98–3.63, P = 0.06), far lateral approach (OR 0.46, 95% Cl 0.21–1.02, P = 0.06), and pediatric patient (OR 2.45, 95% Cl 0.94–6.56, P = 0.07) may be possible predictors for CR, however, they were not statistically significant. NCs are mainly hypodense on CT (32 [61.5%]), varied greatly in intensity on T1WI, hyperintense on T2WI magnetic resonance imaging (98 [67.1%]), and hyperintense on fluid-attenuated inversion recovery (17 [63.0%]).Conclusion: We recommend utilizing various diagnostic imaging tests to help reduce misdiagnoses when identifying intracranial NCs. For patient safety, CR should be achieved when possible, to reduce risk of additional operations due to recurrence.

Bilobed intramedullary and extramedullary bronchogenic cyst of the conus medullaris: illustrative case.

BACKGROUNDSpinal bronchogenic cysts are rare nonneoplastic congenital variants of neurenteric cysts. The natural history and surgical management of these lesions are poorly understood.OBSERVATIONSA 25-year-old male presented with progressive back pain and bilateral lower limb sciatica of 6 months’ duration. He had undergone subtotal resection of an intramedullary bronchogenic conus medullaris cyst 5 years prior. Magnetic resonance imaging revealed a recurrent bilobed intramedullary and extramedullary conus medullaris cystic lesion. The authors resected the lesion via a posterior approach with the aid of intraoperative neuromonitoring. Gross total resection was precluded by the tightly adherent nature of the cyst and the fact that stimulation of a residual intramedullary portion of the lesion evoked external anal sphincter responses.LESSONSThis is the first reported case of a bilobed intramedullary and extramedullary bronchogenic cyst of the conus medullaris. This unique case lends insight into the poorly defined embryogenesis of bronchogenic cysts by favoring the split notochord syndrome theory rather than the ectopic ectoderm proposal. The importance of neuromonitoring when resecting these tightly adherent lesions is demonstrated. Finally, although the secretory nature of these lesions portends a tendency for cyst reaccumulation, it is imperative to recognize that this is usually a slow process.

Clinicopathological Study of Intradural Extramedullary Spinal Tumors and Its Correlation With Functional Outcome.

IntroductionIntradural extramedullary (IDEM) spinal cord tumors account for approximately two-thirds of largely benign intraspinal neoplasms. These are amenable to gross total excision and usually carry a good functional outcome.MethodsIn this study, we reviewed the surgical outcomes of 35 patients who underwent excision of intradural extramedullary tumors. Patient demographics, severity and duration of symptoms, and tumor characteristics (anatomical and pathological) in all operated spinal IDEM tumors were collected. The neurological findings obtained during the preoperative stage and the postoperative follow-up were evaluated according to the Frankel and Nurick grading. The back pain was assessed with help of the Denis pain scale (DPS).ResultsThe histopathological outcomes of the study were as follows: six patients of neurofibroma, 12 cases of schwannoma, nine cases of meningiomas, three cases of ependymoma, one case of dorsal neurenteric cyst, two cases of epidermoid cyst, one case of cauda equina paraganglioma, and one case of filum terminale dermoid cyst. Paresthesia/numbness were the commonest symptoms (88.6%), weakness of limbs in (80%), sphincter dysfunction in 15 patients (42.9%), and paraplegia was seen in three patients (8.57%). The complications encountered were - one case each of cerebrospinal fluid (CSF) leak, surgical site infection, and pseudomeningocele. The percentage of spinal canal occupied ranged from 71-94%. The mean percentage of the spinal canal occupied by the tumor was 81.8%.In our series, 77.14% of patients (p<0.0001) had good functional outcomes as per improvement in Frankel score. The DPS and Nurick score mean values showed a significant decrease over the follow-up duration as compared to preoperative mean values. Significant functional improvement was noted at the one-week, one-month, and one-year follow-up, with a p-value of <0.0001.ConclusionsThe IDEM tumors are usually benign and are readily detected by contrast-enhanced MRI scans. These have excellent surgical outcomes with some exceptions. Greater canal occupancy and a longer duration of symptoms are usually seen to correspond with suboptimal functional outcomes.

Neurenteric Cyst at Craniovertebral Junction: An Uncommon Presentation

Neurenteric cysts, also known as endodermal cysts and enterogenous cysts, are rare benign cysts. Neurenteric cysts may be associated with congenital malformations, such as spinal dysraphism, or may occur as solitary lesions. Most are located in the spine, in the intradural, extramedullary and intraspinal space. Rare examples may occur intracranially, and rarer examples may occur at the craniovertebral junction. Clinically, solitary neurenteric cysts present with mass effect symptoms such as compression of spinal cord and adjacent nerve roots. We present a case of a 28-year-old male patient with a histologically confirmed neurenteric cyst located at the cervicomedullary or craniovertebral junction with presenting symptoms of neck pain and severe debilitating weakness of all four limbs. The patient underwent successful surgical resection, with gradual improvement in his symptoms. We also present a review of literature, including the clinical features, histological characteristics, and management options for patients with neurenteric cyst in the craniovertebral junction.

Endoscopic Endonasal Approaches to the Clival Region.

Our main objectives were to analyze and determine the safety, risk of post-operative complications, and surgical outcome of the endoscopic endonasal approach to the clival region. From May 2011 to May 2019, we operated on 19 patients using the endoscopic endonasal approach to the clival region. Their pathologies were diverse: pituitary macroadenoma, craniopharyngioma, metastasis, and a prepontine neurenteric cyst. The first operations were supervised by an experienced center using telementoring. We explained our surgical technique and analyzed the patients' data, which were included in our study. We managed to achieve complete removal of the pathological process in 14 patients. There were no deaths in the perioperative and early post-operative period. The most common complication was a cerebrospinal fluid leak, which was successfully managed in all of the cases. There were no deaths or significant morbidities in the post-operative period. An endoscopic transnasal approach to the clival region is safe and effective. It provides better visualization of that region compared to other transcranial approaches. The risk of post-operative complications is significantly lower with the help of modern reconstructive techniques. CSF leak is the most frequent complication.

Intracranial Neurenteric Cyst in a Patient with Acute Unilateral Thalamic Infarction.

Neurenteric cysts are a rare type of foregut duplication cyst that are classified as intracranial or spinal and are associated with central nervous system abnormalities.Neurenteric cysts are usually benign and slow-growing, so conservative treatment can be provided if there are no symptoms.Thalamic stroke may be caused by arterial or venous infarction.Cardioembolism is the most common cause of thalamus infarction; variations in blood supply to the thalamus should be investigated in affected patients.

Neurenteric CYST of the craniocervical junction. Case report

Neurenteric cysts are uncommon benign endodermally-derived epithelial-lined cystic lesions of the central nervous system. We report a case of neurenteric cyst in the craniocervical junction and review of the literature.A 17-year-old boy complained of a 9-month history of progressive headaches. Neurological examination did not reveal any abnormalities. A computed tomography scan was performed demonstrating a hypodense lesion in the craniocervical junction and the magnetic resonance imaging (MRI) revealed an approximately 19 × 24 × 23 mm prebulbar cyst that induced posterior displacement of the medulla. Four months later, the patient developed right hemiparesis and limitation of his neck movements. Further MRI showed that the lesion had increased a little in size. Given this, a suboccipital craniotomy was performed with right C1 laminectomy, achieving complete excision of the cyst, and the peribulbar region and the lower cranial nerves were released. After 4 months, he was asymptomatic and playing football again, and 3 years later, MRI did not demonstrate any signs of growth.

Intra-spinal Neurenteric Cyst in an adult- a case report of a rare anomaly

Intra-spinal Neurenteric Cyst in an adult- a case report of a rare anomaly

Resection of a Posterior Fossa Endodermal Cyst With Exoscopic Assistance: 2-Dimensional Operative Video.

Neurenteric cysts are rare benign congenital tumors of endodermal origin that most commonly occur in the cervical and upper thoracic spine, with only about 10% to 18% of the reported cases occurring intracranially.1 A definitive preoperative diagnosis is complicated by the variable appearance of neurenteric cysts on magnetic resonance (MR) imaging.2 The recommended treatment of neurenteric cysts is complete surgical resection when possible.3,4 We present a case of a posterior fossa neurenteric cyst. A 33-yr-old man without medical history presented with left-sided headache and mild left-sided facial numbness and weakness. Admission MR imaging revealed a nonenhancing mass, which was hyperintense on T1-weighted MR images, compressing the brainstem anteriorly. The lesion was isointense on T2 FLAIR images and hypointense on diffusion-weighted imaging, initially read as possible epidermoid cyst. The patient underwent a left-sided retrosigmoid craniotomy via far lateral transcondylar approach. The tumor was adjacent to both vertebral arteries, the left PICA, and cranial nerves (CN) VII-XII with superior extension to CN V. The cyst was encased in a thin capsule, and its contents were yellowish in color and ranged from thick liquid to colloidal and caseous consistency. The cyst also contained heavily calcified portions, which were excised using sharp dissection. Images of the cyst wall show that it is focally lined with ciliated columnar epithelium with intracellular mucin confirming an endodermal or neurenteric cyst. After the operation, the patient's symptoms resolved, and he was discharged on postoperative day 4. Postoperative MR images confirmed gross total resection. The patient consented to video production.

Surgical anatomy of a neurenteric cyst anterior to the brainstem

The authors report on a sixteen-month old boy who presented a neurenteric cyst located in the cervicomedullary junction, anterior to the brain stem. Centred on the ventral aspect of the premedullary cistern, the cyst extended from the prepontine space to the cervicomedullary junction, causing important brain stem compression. The lesion was totally removed via a posterior approach with suboccipital craniotomy and laminectomy of C1. After surgery the patient recovered his neurological function and the postoperative MRI showed total resection of the cyst.

Neurenteric cysts at foramen magnum in children: presentation, imaging characteristics, and surgical management-case series and literature review.

Neurenteric cysts (NEC) are rare benign tumors of the central nervous system. Even more rare are the NEC at the ventral foramen magnum in children that are misdiagnosed. They cause distortion of the vertebrobasilar vascular system and cervicomedullary neural structures and are adherent to the latter. The inferior portion lies ventral to the upper most dentate ligament. This has implications in the surgical approach for complete excision. We reviewed our series of five children with ventral foramen magnum NEC in the MRI era and analyzed presentation, radiological findings and surgical management. A literature review is also presented. There were two females and five males. Headache and neurological deficit was present in all five; one child presented with recurrent meningitis. The NEC was present ventral to the cervicomedullary junction in all cases. The posterolateral transcondylar approach was used for complete excision with no recurrences. Neurenteric cysts at the ventral foramen magnum can be confused with other cystic lesions. Headaches are the most common presentation. The posterolateral transcondylar approach to the ventral cervicomedullary junction provides the most reliable avenue for entire resection.

Therapeutic Strategies and Prognostic Factors Based on 121 Spinal Neurenteric Cysts.

Limited data existed to guide the management of intraspinal neurenteric cysts (ISNECs). To evaluate the risk factors for progression-free survival (PFS), elucidate the radiological features of ISNECs, and propose a treatment protocol. From 2003 to 2015, 121 patients with pathologically confirmed ISNECs treated at our institute were included in this study. Pertinent risk factors were evaluated. Gross total resection (GTR) was achieved in 55 (44.6%) patients; 106 (87.6%), 12 (9.9%), and 3 (2.5%) ISNECs were classified as Wilkins A, B, and C, respectively. After a median follow-up duration of 64.2 mo, recurrence occurred in 25 (22.7%) patients, with a median PFS time of 43.1 mo. The actuarial PFS rates at 5 and 10 yr were 73.2% and 66.2%, respectively. The actuarial overall survival rates at 5 and 10 yr were 100% and 97.6%, respectively. Non-GTR (hazard ratio [HR], 5.836; 95% confidence interval [CI], 1.698-20.058; P=.005), Wilkins B/C (HR, 3.129; 95% CI, 1.009-9.702; P=.048), and a history of surgical resection (HR, 3.690; 95% CI, 1.536-8.864; P=.004) were adverse factors. GTR and Wilkins A were favorable factors for PFS. If tolerable, GTR alone was advocated as an optimal treatment. Because of the benign nature and favorable prognosis, non-GTR was an alternative if GTR failed. Close follow-up was needed because of the recurrent tendency of ISNEC. Future study with a large cohort is necessary to verify our findings.

Lumbar Intradural Extramedullary Isolated Hemagioblastoma: A Rare Entity and Review of Literature

The terminology “Intradural Extramedullary” is often associated with Nerve sheath tumors (schwannoma, Neurofibroma) and Meningioma, while dermoid cyst, epidermoid, neurenteric cyst are other benign entities. Hemangioblastomas are rare, accounting for 1-5% of all spinal cord tumours.1 75% of the Spinal hemangiblastoma are Intramedullary, and another 10-15% have combined intramedullary and extramedullary-intradural component, while isolated extramedullary hemangioblastoma are rare1. Our case had an isolated intradural extramedullary hemangioblastoma without any associated manifestations of VHL syndrome. Surgery is a preferred option where total resection is possible, where as SRS is also an option in case of residual or incomplete resection.

A RARE CASE OF NEUROENTERIC CYST INVOLVING THE ANTERIOR FOSSA; REPORT OF A CASE AND REVIEW OF LITERATURE

Neuroenteric cysts, are rare benign endodermal lesions which mostly occur in the central nervous system. We report a case of a neuroenteric cyst in a 30-year-old man who presented with rhinoliquorrhea at the ENT department. After clinical examination, a semisolid mass was revealed in the left nostril. The Computer Tomography Scan revealed a frontal ethmoidal nasal meningoencephalocele with inferior extension into the left nostril. Gadolinium-enhanced T1-weighted MR images showed a well-defined frontal mass with ring-like enhancement and extension into the cribriform plate of the ethmoid bone and into the left nostril. The lesion measured 10/10/20 mm. The tumor was totally resected using a unilateral subfrontal approach. At five months’ follow-up, the patient showed significant amelioration of symptoms and remission of cerebrospinal fluid leakage. Native and Contrast-enhanced Cerebral Computer Tomography, as well as Magnetic Resonance Imaging showed total surgical resection of the cyst. Supratentorial neurenteric cysts involving the anterior fossa are rare. Intracranial neurenteric cysts should be differentiated by any well-demarcated cystic tumors. The gold standard treatment remains complete surgical resection with favorable outcome.

CS-07 A CYSTIC LONG-SEGMENT CERVICAL SCHWANNOMA: A CASE REPORT

Schwannomas are the most common intradural extramedullary spinal tumors. However, they are usually solid tumors, and totally cystic changes are rare. Herein, we report a case of a 46-year-old male presenting with numbness of right limbs, right hemiplegia, and posterior neck pain for one year. MRI revealed a well-defined cystic long-segment, from C1 to C6, intradural extramedullary mass. The lesion showed hypointense on T1WI, hyperintense on T2WI, hyperintense on DWI, and it was marginally enhanced on the contrast image with Gd-DTPA. C1 laminectomy and hemi-laminectomy from C2 to C6 was performed for tumor resection. The tumor was found to be totally cystic and tensed with a jelly-like content. It was completely resected with the attachment of the C3 dorsal root. Histopathological examination confirmed it to be a schwannoma. The mechanism of cyst formation in schwannoma is considered as results of ischemic necrosis associated with tumor growth, or cystosis due to degeneration of Antoni-type B region. The long-segment, totally cystic intradural cervical schwannoma is rare, but it should be included in the differential diagnosis of a cystic mass in the spinal region. It can be difficult to distinguish cystic spinal schwannomas from other cystic lesions like arachnoid cyst, epidermoid cyst, and neurenteric cyst. Contrast enhanced MRI is useful by enhancing the margin of the tumor.

CS-03 LARGE CYSTIC INTRADURAL SCHWANNOMA IN CERVICAL REGION: A CASE REPORT

Schwannomas are the most common intradural extramedullary spinal tumors. However, they are usually solid tumors, and totally cystic changes are rare. Herein, we report a case of a 46-year-old male presenting with numbness of right limbs, right hemiplegia, and posterior neck pain for one year. MRI revealed a well-defined cystic long-segment, from C1 to C6, intradural extramedullary mass. The lesion showed hypointense on T1WI, hyperintense on T2WI, hyperintense on DWI, and it was marginally enhanced on the contrast image with Gd-DTPA. C1 laminectomy and hemi-laminectomy from C2 to C6 was performed for tumor resection. The tumor was found to be totally cystic and tensed with a jelly-like content. It was completely resected with the attachment of the C3 dorsal root. Histopathological examination confirmed it to be a schwannoma. The mechanism of cyst formation in schwannoma is considered as results of ischemic necrosis associated with tumor growth, or cystosis due to degeneration of Antoni-type B region. The long-segment, totally cystic intradural cervical schwannoma is rare, bit it should be included in the differential diagnosis of a cystic mass in the spinal region. It can be difficult to distinguish cystic spinal schwannomas from other cystic lesions like arachnoid cyst, epidermoid cyst, and neurenteric cyst. Contrast enhanced MRI is useful by enhancing the margin of the tumor.

Recurrent neurenteric cysts compressing the brainstem.

Background:Neurenteric cysts, also referred to as endodermal cysts and enterogenous cysts, are rare lesions of the neuroaxis occurring most frequently within the spinal cord and rarely intracranially. In the literature describing these lesions, examples of intraoperative imaging and cytology preparations are rare to non-existent.Case Description:Here, we describe a case of a recurrent posterior fossa neurenteric cyst compressing the brainstem in a 47-year-old female and causing incontinence and progressive quadriparesis. Intraoperative findings and cytologic and histologic features are presented.Conclusion:Neurenteric cysts are generally considered to be benign and slow-growing though recurrence is common. This case of a recurrent neurenteric cyst is illustrated by intraoperative macroscopic and cytologic images.